prednisone and Granulomatosis, Wegener's studies

Prednisone: A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.
prednisone : A synthetic glucocorticoid drug that is particularly effective as an immunosuppressant, and affects virtually all of the immune system. Prednisone is a prodrug that is converted by the liver into prednisolone (a beta-hydroxy group instead of the oxo group at position 11), which is the active drug and also a steroid.

Research Excerpts

Excerpt	Relevance	Reference
"Current standard therapy for Wegener's granulomatosis and microscopic polyangiitis combines corticosteroids and cyclophosphamide to induce remission, followed by a less toxic immunosuppressant such as azathioprine or methotrexate for maintenance therapy."	9.13	Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. ( Boffa, JJ; Cohen, P; Cordier, JF; Ducroix, JP; Guillevin, L; Hamidou, MA; Khellaf, M; Kyndt, X; Lambert, M; Le Noach, J; Lifermann, F; Mahr, A; Merrien, D; Mouthon, L; Pagnoux, C; Papo, T; Puéchal, X; Ruivard, M; Vinzio, S, 2008)
"It has been recognized that nasal cocaine abuse can induce midline destructive lesions that can mimic different disorders, including small-vessel vasculitis."	7.79	Vasculitis mimics: cocaine-induced midline destructive lesions. ( Espinoza, LR; Perez Alamino, R, 2013)
"Patients with microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) received either avacopan 30 mg twice daily for 52 weeks plus prednisone-matching placebo or tapered prednisone over 20 weeks plus avacopan-matching placebo for 52 weeks."	5.69	Efficacy and safety of avacopan in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis: A subanalysis of a randomized Phase 3 study. ( Dobashi, H; Harigai, M; Kaname, S; Kubono, S; Tamura, N; Yoshida, T, 2023)
"PC recruitment and Web-based informed consent was compared with CoE recruitment for a randomized controlled trial (RCT) of continuing versus stopping low-dose prednisone for maintenance of remission of patients with granulomatosis with polyangiitis (GPA)."	5.24	Experience With Direct-to-Patient Recruitment for Enrollment Into a Clinical Trial in a Rare Disease: A Web-Based Study. ( Borchin, R; Burroughs, C; Carette, S; Cronholm, PF; Davis, T; Easley, E; Khalidi, N; Koening, C; Krischer, J; Kullman, J; Langford, CA; McAlear, CA; Merkel, PA; Monach, P; Moreland, L; Pagnoux, C; Specks, U; Sreih, AG; Ytterberg, S, 2017)
"Current standard therapy for Wegener's granulomatosis and microscopic polyangiitis combines corticosteroids and cyclophosphamide to induce remission, followed by a less toxic immunosuppressant such as azathioprine or methotrexate for maintenance therapy."	5.13	Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. ( Boffa, JJ; Cohen, P; Cordier, JF; Ducroix, JP; Guillevin, L; Hamidou, MA; Khellaf, M; Kyndt, X; Lambert, M; Le Noach, J; Lifermann, F; Mahr, A; Merrien, D; Mouthon, L; Pagnoux, C; Papo, T; Puéchal, X; Ruivard, M; Vinzio, S, 2008)
" The fourth month of induction therapy with cyclophosphamide and methylprednisone she presented with dyspnea and respiratory failure."	3.88	[EBV-ASSOCIATED PNEUMONIA IN PATIENT WITH GRANULOMATOSIS WITH POLYANGIITIS (GPA) IN IMMUNOSOPPRESSIVE THERAPY TREATED WITH ACICLOVIR]. ( Boscutti, G; Bregant, C; Carraro, M; Di Maso, V; Ermacora, E; Lorenzon, E; Mongera, N; Pian, M; Savi, U, 2018)
"It has been recognized that nasal cocaine abuse can induce midline destructive lesions that can mimic different disorders, including small-vessel vasculitis."	3.79	Vasculitis mimics: cocaine-induced midline destructive lesions. ( Espinoza, LR; Perez Alamino, R, 2013)
"Eighty-five patients with Wegener's granulomatosis were studied for 21 years at the National Institutes of Health."	3.75	Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. ( Fauci, AS; Haynes, BF; Katz, P; Wolff, SM, 1983)
" Three groups of patients were prospectively evaluated: Group I: 15 patients with Wegener's granulomatosis and 4 patients with severe scleritis received a single dose cyclophosphamide (15 mg/kg bw/day) and 250 mg prednisone i."	3.70	Unique alterations of thyroid function parameters after i.v. administration of alkylating drugs (cyclophosphamide and ifosfamide). ( Jockenhövel, F; Kummer, G; Mann, K; Philipp, T; Reinhardt, W; Reinwein, D; Sauter, V; Uppenkamp, M; Witzke, O, 1999)
"A 44-year-old man with Wegener's granulomatosis involving the upper and lower respiratory tracts developed a diplopia with involvement of three extraocular muscles of one eye and one extraocular muscle of the other eye."	3.66	Extraocular muscle involvement in Wegener's granulomatosis. ( Bergstrom, TJ; Pinchoff, BS; Sandall, GS; Spahlinger, DA, 1983)
"The authors report a case of a patient with an ENT granuloma, treated successfully with cyclophosphamide and prednisone in 1976."	3.66	[Tumorous form of Wegener's granulomatosis following a malignant facial granuloma. Diagnostic value]. ( Houssin, A; Lavenet, F; Penneau, D; Saint-André, JP; Thomas, B, 1983)
" Oral corticosteroid dose, adverse events, exacerbations, symptom control, disease activity, blood markers, and lung function were evaluated before, during, and after 7 monthly reslizumab treatments."	3.01	Efficacy and safety of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis. ( Denson, JL; Dollin, YT; Dunn, RM; Guntur, VP; Manka, LA; Strand, MJ; Wechsler, ME, 2021)
"A prednisone increase led to remission in 35 patients (80%)."	2.80	Outcomes of nonsevere relapses in antineutrophil cytoplasmic antibody-associated vasculitis treated with glucocorticoids. ( Brunetta, P; Ding, L; Fervenza, FC; Hoffman, GS; Iklé, D; Kallenberg, CG; Langford, CA; Lim, N; Merkel, PA; Miloslavsky, EM; Monach, PA; Seo, P; Specks, U; Spiera, R; St Clair, EW; Stone, JH; Tchao, NK; Villareal, M, 2015)
"Prednisone was tapered and discontinued over 5 mo."	2.72	Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. ( Carlson, KA; Fervenza, FC; Keogh, KA; Schroeder, DR; Specks, U; Ytterberg, SR, 2006)
"Patients with Wegener's granulomatosis or microscopic polyangiitis or Churg-Strauss syndrome and one or more poor prognostic factors must receive a combination of corticosteroids and immunosuppressants, mainly intravenous pulsed cyclophosphamide."	2.44	[Treatment of ANCA-associated vascularitides]. ( Guillevin, L; Pagnoux, C, 2007)
"Strawberry gingivitis is the most common oral manifestation and is characteristic."	2.43	Wegener's granulomatosis: the current understanding. ( Kumaran, MG; Ponniah, I; Shaheen, A; Shankar, KA, 2005)
"When unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians."	2.42	Vasculitis of the nervous system. ( Younger, DS, 2004)
"In cases of Wegener's granulomatosis with salivary symptoms, measurement of salivary gland functional capacity, in addition to the c-ANCA test, may help to monitor disease activity."	2.39	Salivary gland involvement in Wegener's granulomatosis. A case report and review of the literature. ( Lustmann, J; Markitziu, A; Segal, N, 1994)
"The clinical manifestations of Wegener's granulomatosis (WG) may be varied and easily overlooked."	2.39	Wegener's granulomatosis: case report and review of the literature. ( Ataman, M; Gürsel, B; Sarioglu, T; Shahidi, H, 1994)
"Wegener's granulomatosis is a multisystem life threatening vasculitis which poses particular concerns when it involves a pregnant patient."	2.39	Wegener's granulomatosis complicating pregnancy: presentation of two patients and review of the literature. ( Abrons, HL; Habib, A; MacKay, K, 1996)
"The response of Wegener's granulomatosis to appropriate therapy can be dramatic, whereas without treatment it is a progressive and mutilating disorder with life-threatening complications."	2.38	Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review. ( Burlacoff, SG; Wong, FS, 1993)
"Wegener's granulomatosis is a rare disease of unknown etiology."	2.38	Wegener's granulomatosis. ( Garrison, KL; Geiger, WJ; Losh, DP, 1992)
"Midline granuloma (MG), limited Wegener's granulomatosis (LWG), and generalized Wegener's granulomatosis (WG) have been viewed by some investigators as representing an interrelated disease spectrum."	2.35	Midline granuloma and Wegener's granulomatosis: clinical & therapeutic considerations. ( Bole, GG; Schechter, SL; Walker, SE, 1976)
"Prednisone was reduced to 50 mg/d 1 month thereafter."	1.91	ANCA-negative eosinophilic granulomatosis with polyangiitis complicated by peripheral nerve damage: A case report. ( Chen, Y; Liu, B; Wan, Q, 2023)
"Other comorbidities included bronchial asthma, chronic sinusitis, ischemic heart disease, mild aortic stenosis, arterial hypertension, and degenerative thoracic spine disease."	1.72	Torasemide-induced Vascular Purpura in the Course of Eosinophilic Granulomatosis with Polyangiitis. ( Bergler-Czop, B; Frątczak, A; Miziołek, B; Polak, K, 2022)
"Aortitis and pachymeningitis are uncommon manifestations of the antineutrophil cytoplasmic antibody-associated vasculitides, a group of systemic autoimmune diseases mainly affecting small vessels."	1.51	Aortitis and pachymeningitis: an unusual combination in granulomatosis with polyangiitis (myeloperoxidase-associated vasculitis). ( Abdulqader, Y; Parperis, K, 2019)
" During omalizumab treatment, spirometry, the asthma control test (ACT) score and eosinophilia were evaluated, and prednisone dosage was recorded."	1.43	Omalizumab in patients with eosinophilic granulomatosis with polyangiitis: a 36-month follow-up study. ( Detoraki, A; Di Capua, L; Genovese, A; Marone, G; Spadaro, G; Varricchi, G, 2016)
"Granulomatosis with polyangiitis (Wegener's granulomatosis) is one of the idiopathicimmune-mediated small-vessel vasculitides described in humans which are characterised by the presence of circulating antineutrophil cytoplasmic antibodies."	1.42	Erosive rhinitis resembling granulomatosis with polyangiitis (Wegener's granulomatosis) in an Anatolian shepherd dog. ( Basson, S; Böhm, M, 2015)
"Rituximab is used for the treatment of granulomatosis with polyangiitis (GPA), historically known as Wegener's granulomatosis."	1.40	Otolaryngological progression of granulomatosis with polyangiitis after systemic treatment with rituximab. ( Kim, J; Kim, YJ; Malm, IJ; Mener, DJ; Seo, P, 2014)
"A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response."	1.40	[C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab]. ( Aldasoro-Cáceres, I; Aldasoro-Cáceres, V; Ibáñez-Bosch, R; Murié-Fernández, M; Pérez-Moreiras, JV, 2014)
"We present 2 cases where central diabetes insipidus was the presenting manifestation of GPA."	1.40	Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis. ( Al-Fakhouri, A; Gan, J; Manadan, A; Sreih, AG, 2014)
"Immune-mediated scleritis is a rare condition, and the information on the clinical course and complications is scarce."	1.40	Five-year outcome in immune-mediated scleritis. ( Bernauer, W; Brunner, M; Pleisch, B, 2014)
"The diagnosis of Wegener's granulomatosis was based on the association of positive antineutrophil cytoplasmic antibodies, urinary sediment abnormalities and renal biopsy findings."	1.35	[Pulmonary fibrosis as a presentation of Wegener's granulomatosis]. ( Abid, T; Ayoub, A; Boudawara, T; Gharsalli, H; Kammoun, K; Ketata, W; Makni, S; Mrabet, S; Msaad, S, 2009)
"Respiratory obstruction in Wegener granulomatosis can result from subglottic stenosis."	1.35	[Subglottic stenosis in Wegener's granulomatosis]. ( Bakhos, D; Beutter, P; Diot, E; Lescanne, E; Morinière, S, 2008)
" During year 1, the mean cumulative prednisone dosage in the multiple flares subgroup was 7."	1.35	Effects of glucocorticoids on weight change during the treatment of Wegener's granulomatosis. ( Anderson, T; Davis, JC; Fontaine, KR; Hoffman, GS; McCune, WJ; Merkel, PA; Specks, U; Spiera, R; St Clair, EW; Stone, JH; Wung, PK, 2008)
"Serositis was resolved in both cases."	1.34	[Rituximab for treatment of patients with systemic autoimmune diseases]. ( Castillo Palma, MJ; Colorado Bonilla, R; García Hernández, FJ; Garrido Rasco, R; González León, R; Ocaña Medina, C; Sánchez Román, J, 2007)
"Conventional therapy for Wegener's granulomatosis, steroid and cyclophosphamide, fails to control disease activity in some refractory patients and has treatment-related toxicity."	1.34	Two cases of refractory Wegener's granulomatosis successfully treated with rituximab. ( Hashimoto, H; Hirashima, M; Ikeda, M; Kaneda, K; Kobayashi, S; Matsudaira, R; Morimoto, S; Nawata, M; Tajima, M; Takasaki, Y; Tamura, N, 2007)
"Glomerulonephritis was present in 22 patients at presentation."	1.34	Clinical features and outcome of pediatric Wegener's granulomatosis. ( Akikusa, JD; Harvey, EA; Hebert, D; Laxer, RM; Schneider, R; Silverman, ED; Thorner, PS, 2007)
"Wegener granulomatosis is a rare, chronic, multisystemic vasculitis affecting mainly the upper and lower respiratory tracts together with glomerulonephritis, but the disease may involve any other organ."	1.34	Protracted superficial Wegener granulomatosis in a child. ( Ben Ghorbel, I; Dhrif, AS; Houman, MH; Miled, M, 2007)
"Diagnosis of Wegener's granulomatosis (WG) can be delayed because of its aspecific presenting symptoms."	1.33	Wegener's granulomatosis: a challenging disease for otorhinolaryngologists. ( Agostino, S; Cadoni, G; Calŏ, L; Campobasso, E; Manna, R; Paludetti, G; Prelajade, D, 2005)
"We describe a patient with Wegener's granulomatosis (WG) who developed neurological symptoms attributed to meningeal involvement."	1.33	Meningeal involvement in Wegener's granulomatosis. ( Al Dhanhani, A; Hanly, JG; Macaulay, R; Maloney, B, 2006)
"Generalized or systemic Wegener granulomatosis has a worse prognosis than those limited to the respiratory tracts."	1.32	A case report of wegener granulomatosis treated only with corticosteroids for 30 years. ( Cockcroft, DW; PausJenssen, ES, 2003)
"Wegener's granulomatosis is a systemic vasculitis of unknown etiology, primarily affecting the upper and lower respiratory tract and the kidneys, although there is a form restricted to the lungs."	1.32	[Polyradiculitis and Wegener's granulomatosis]. ( Ancochea, J; Casanova, A; Girón, RM; Pérez Amor, E; Vélez, MD; Zamora, E, 2004)
"A diagnosis of seronegative Wegener's granulomatosis was made and the patient received a combination of prednisone and cyclophosphamide with clinical improvement and clearance of the radiological lesions in the lungs."	1.32	Rapidly progressive diffuse large B-cell lymphoma with initial clinical presentation mimicking seronegative Wegener's granulomatosis. ( Amariglio, N; Amir, G; Cohen, Y; Polliack, A; Schibi, G, 2004)
"In order to study the characteristic of Wegener granulomatosis in otorhinolaryngology."	1.32	[The feathers of Wegener granulomatosis in otorhinolaryngology]. ( Li, H; Li, X; Shang, Y, 2004)
"We report a case of severe Wegener's granulomatosis that caused end-stage renal failure and recurrent flares of sinusitis and pulmonary manifestations."	1.31	Control of a relapse and induction of long-term remission of Wegener's granulomatosis by cyclosporine. ( Fournier, A; Ghez, D; Henry, I; Lassoued, K; Pruna, A; Westeel, PF, 2002)
"Eighteen patients (12 female) with Wegener's granulomatosis (WG) treated from 1982-2001 were studied."	1.31	[Wegener's granulomatosis--disease course and treatment response of 18 patients]. ( Bestry, I; Demkow, U; Korzeniewska-Koseła, M; Kuś, J; Langfort, R; Maziarka, D; Sobiecka, M, 2002)
"Granulomatous Pneumocystis carinii pneumonia developed under immunosuppressive treatment with cyclophosphamide and prednisone."	1.31	Granulomatous Pneumocystis carinii pneumonia in Wegener's granulomatosis. ( Binet, I; Dalquen, P; Ebnöther-Staub, C; Mayr, M; Solèr, M; Tamm, M; Ullmer, E, 2000)
"The authors present a case of Wegener's granulomatosis in a 22 year-old female patient."	1.31	[Wegener's granulomatosis]. ( Ellert-Zygadłowska, J; Radowska, D, 2000)
"We report a case of Wegener's granulomatosis (WG) with pulmonary hemorrhage also satisfying the criteria for antiphospholipid antibody syndrome (APS)."	1.31	Wegener's granulomatosis associated with antiphospholipid syndrome. ( Castellino, G; La Corte, R; Santilli, D; Trotta, F, 2000)
"Wegener relapse was diagnosed and the combination of three methylprednisolone perfusions followed by oral prednisone (1 mg/kg/d) and a monthly bolus of cyclophosphamide led to a new remission."	1.31	Relapse of Wegener's granulomatosis. Concerning a case after 20 years of remission. ( Bourgeois, P; de Person, JF; de Sauverzac, C; Fautrel, B; Foltz, V; Koeger, AC, 2001)
"This is the first reported case of Wegener's granulomatosis presenting with an isolated tubulointerstitial lesion."	1.31	Wegener's granulomatosis presenting as acute suppurative interstitial nephritis. ( Banerjee, A; Farrington, K; McKane, W; Thiru, S, 2001)
"The cases of three patients with Wegener's granulomatosis presenting as exophthalmos and cranial neuropathy are reported."	1.30	[Exophthalmos and cranial neuropathy as a form of presentation of Wegener's granulomatosis]. ( Brieva, L; Campello, I; Hermida, I; Losfablos, F; Sanjuán, F; Velilla, J, 1997)
"Despite numerous symptoms suggesting Wegener's granulomatosis, ineffective treatment had been instituted for two months."	1.30	[Wegener's granulomatosis]. ( Nalepa, P; Niemiec, A, 1997)
"Although rare, Wegener's granulomatosis should be included in the differential diagnosis of chronic aseptic meningitis, communicating hydrocephalus, and papilledema."	1.30	Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener's granulomatosis: case report and literature review. ( Medsger, TA; Scarrow, AM; Segal, R; Wasko, MC, 1998)
"Disseminated histoplasmosis is an unusual complication in endemic areas and has been reported in patients with an immunodeficient state, either from human immunodeficiency virus (HIV) infection or transplantation."	1.30	Disseminated histoplasmosis and Wegener's granulomatosis. ( Manepalli, AN; Rush, L, 1998)
"We report 8 patients with Wegener granulomatosis (WG) who suffered from symptomatic urogenital involvement including acute urinary retention related to prostatitis, orchitis, ureteral stenosis, bladder pseudotumor, and penile ulceration."	1.29	Urogenital manifestations of Wegener granulomatosis. ( Bletry, O; Godeau, P; Huong, DL; Papo, T; Piette, JC; Richard, F; Valcke, JC; Wechsler, B, 1995)
"A patient with Wegener's granulomatosis is reported."	1.29	Urethral stricture as unusual complications of Wegener's granulomatosis. ( Borkowski, A; Droszcz, W; Madalinska, M; Pogorzelski, R; Zielonka, TM, 1995)
"carinii pneumonia in patients with Wegener's granulomatosis."	1.29	Pneumocystis carinii pneumonia: a major complication of immunosuppressive therapy in patients with Wegener's granulomatosis. ( Fauci, AS; Hoffman, GS; Kerr, GS; Leavitt, RY; Ognibene, FP; Reda, D; Shelhamer, JH, 1995)
"The pattern of relapses is described in a consecutive series of 150 patients with vasculitis."	1.29	Necrotizing vasculitis--relapse despite cytotoxic therapy. ( Adu, D; Bacon, PA; Emery, P; Gordon, M; Greaves, I; Luqmani, RA; Michael, J; Richards, N, 1993)
"We observed systematic vasculitis (polyarteritis nodosa and Wegener's granulomatosis) in several members of 2 different families."	1.29	Familial vasculitis: report of 2 families. ( Cotch, MF; Fauci, AS; Hoffman, GS; Rottem, M, 1994)
"Wegener's granulomatosis is a well-defined systemic vasculitic syndrome that primarily affects the upper and lower respiratory tracts and the kidney."	1.29	Case report: Hashimoto's thyroiditis associated with Wegener's granulomatosis. ( Gal, AA; LiVolsi, VA; Masor, JJ, 1994)
"A kidney biopsy was consistent with Wegener's granulomatosis, and treatment with prednisone and cyclophosphamide was associated with normalization of serum calcium levels, improved renal function, a marked decrease in serum 1,25(OH)2D levels, and increased serum intact parathyroid hormone levels."	1.29	Case report: hypercalcemia with inappropriate 1,25-dihydroxyvitamin D in Wegener's granulomatosis. ( Findling, JW; Redlin, KC; Shaker, JL; Warren, GV, 1994)
"Two new cases of Wegener's granulomatosis are reported."	1.29	[Wegener's disease: localized and generalized forms]. ( Abenia Ingalaturre, JM; Cámara Jiménez, F; Galve Royo, A; Lahoz Zamarro, MT; Valero Ruiz, J, 1994)
"Wegener's granulomatosis was diagnosed on the basis of suggestive thoracic imaging, microhematuria and necrotizing arteritis with granulomas in muscular biopsy."	1.29	[Inflammatory myopathy: unusual presentation of a case of Wegener disease]. ( Aubert, J; Guelpa, G; Mantegani, A, 1993)
"Five patients with active Wegener's granulomatosis were treated with the immunosuppressive agent Cyclosporin A, along with low dose prednisone."	1.29	Cyclosporin A therapy for Wegener's granulomatosis. ( Allen, NB; Caldwell, DS; McCallum, RM; Rice, JR, 1993)
"A patient is described in whom a recurrence of vasculitis was observed in the kidney allograft six years after transplantation and one year after withdrawal of corticosteroid therapy."	1.29	Late recurrence of systemic vasculitis after kidney transplantation involving the kidney allograft. ( Allegri, L; Banfi, G; Bignardi, L; Fogazzi, GB, 1993)
"Eleven patients with generalized Wegener's granulomatosis were studied."	1.29	Radiographic course of pulmonary manifestations in Wegener's granulomatosis under immunosuppressive therapy. ( Grotz, W; Mundinger, A; Peter, HH; Schollmeyer, P; Würtemberger, G, 1994)
"A patient with Wegener's granulomatosis developed a systemic cytomegalovirus infection that mimicked an exacerbation of her vasculitis including pulmonary infiltrates, renal insufficiency, and cutaneous necrotizing ulcers."	1.29	Systemic cytomegalovirus infection mimicking an exacerbation of Wegener's granulomatosis. ( Greenfield, JW; McCune, WJ; O'Rourke, KS; Weiss, DJ, 1993)
"Most aspects of Wegener granulomatosis were similar in childhood-onset and adult-onset patients."	1.29	Wegener granulomatosis in children and adolescents: clinical presentation and outcome. ( Fauci, AS; Hallahan, CW; Hoffman, GS; Kerr, GS; Leavitt, RY; Lebovics, R; Rottem, M, 1993)
"Among 66 patients with Wegener's granulomatosis, 9 had an intra-alveolar haemorrhage which revealed the disease."	1.29	[Intra-alveolar hemorrhage in Wegener's granulomatosis. Retrospective study of 9 cases]. ( Blétry, O; Godeau, P; Lê Thi Huong, Du; Papo, T; Piette, JC; Sauvaget, F; Wechsler, B, 1993)
"To determine if elderly patients with Wegener's granulomatosis (WG) exhibit distinctive clinical features or outcomes compared with patients whose conditions were diagnosed at younger ages."	1.29	Wegener's granulomatosis in the elderly. ( Covin, RB; Krafcik, SS; Lynch, JP; Sitrin, RG, 1996)
"We describe three children with Wegener granulomatosis who responded well to treatment with glucocorticoids and methotrexate, similar to a regimen used in adults."	1.29	Methotrexate treatment of Wegener granulomatosis in children. ( Gottlieb, BS; Ilowite, NT; Miller, LC, 1996)
"We describe a 3 patients with Wegener's granulomatosis in remission while taking cyclophosphamide and prednisone who developed upper airway obstruction."	1.29	[Subglottic tracheal stenosis in Wegener's granulomatosis. Report of 3 cases]. ( Dobrzyński, P; Pogorzelski, R; Zielonka, TM, 1996)
"One patient each had polyarteritis nodosa, Wegener's granulomatosis, bronchiolitis obliterans with organizing pneumonia, and chronic lymphocytic leukemia with red blood cell aplasia."	1.28	Pulmonary complications of combination therapy with cyclophosphamide and prednisone. ( Brock, N; Fisher, W; Sen, RP; Walsh, TE, 1991)
"The diagnosis of a Wegener's granulomatosis-microscopic polyarteritis disorder was made on the basis of clinical presentation and a positive anti-neutrophil cytoplasmic autoantibody (ANCA)."	1.28	Anti-neutrophil cytoplasmic autoantibodies in a child with pauci-immune necrotizing and crescentic glomerulonephritis. ( Baldree, LA; Gaber, LW; McKay, CP, 1991)
"Of the 180 patients with Wegener granulomatosis referred to the National Institute of Allergy and Infectious Diseases during the past 24 years, 158 have been followed for 6 months to 24 years (a total of 1229 patient-years)."	1.28	Wegener granulomatosis: an analysis of 158 patients. ( Fauci, AS; Hallahan, CW; Hoffman, GS; Kerr, GS; Leavitt, RY; Lebovics, RS; Rottem, M; Travis, WD, 1992)
"Five cases of Wegener's granulomatosis treated with cyclophosphamide (CPM) and prednisolone are reported."	1.28	[Wegener's granulomatosis. Therapeutic indications and follow-up course. Apropos of 5 cases]. ( Allard, P; Bréchot, JM; Capron, F; Lebeau, B; Prudent, J; Rochemaure, J; Urban, T, 1991)
"The Wegener's granulomatosis is a rare primary systemic vasculitis."	1.28	[Clinical and histomorphologic findings in Wegener's granulomatosis]. ( Eckert, H; Fischer, P, 1991)
"Two cases of Wegener's granulomatosis are presented."	1.28	[Wegener's granulomatosis. The value of correct diagnosis and treatment]. ( Biernacki, J; Dobrzyński, P; Droszcz, W; Osuch-Wójcikiewicz, E; Zielonka, TM, 1991)
"Fourteen patients with Wegener's granulomatosis (WG) and severe renal and extrarenal involvement were studied (serum creatinine on admission 5."	1.28	Crescentic glomerulonephritis in Wegener's granulomatosis: morphology, therapy, outcome. ( Böhler, J; Grotz, W; Keller, E; Peter, HH; Rohrbach, R; Schollmeyer, P; Wanner, C, 1991)
"We report here a 41-year-old woman with Wegener's granulomatosis, in whom renal transplantation was performed after a 10-month period of hemodialysis during which no signs of disease activity were detected."	1.28	Hemodialysis does not always protect against recurrence of Wegener's granulomatosis. ( Aunsholt, NA; Pedersen, RS, 1990)
"Although the mouth is often involved in Wegener granulomatosis, oral lesions as the initial sign are rare."	1.28	Oral presentation of Wegener granulomatosis. ( Glass, EG; Lawton, LR; Truelove, EL, 1990)
"Most patients with Wegener's granulomatosis initially present with upper airway symptoms, persistent rhinosinusitis or otitis media."	1.28	[Subglottic stenosis: unrecognized type of presentation of Wegener's granulomatosis]. ( Dudler, J; Fellay, G; Regamey, C, 1990)
"A diagnosis of Wegener's granulomatosis was entertained."	1.27	Wegener's granulomatosis in a 7 year old child. ( O'Regan, S; Sinnassamy, P, 1984)
"In 4 consecutive cases of Wegener's granulomatosis with glomerulonephritis, antibodies against the extractable nuclear antigen SSA (Ro) could be demonstrated with counterimmunoelectrophoresis."	1.27	SSA (Ro)-antibodies in Wegener's granulomatosis. ( Andrassy, K; Darai, G; Koderisch, J; Ritz, E, 1983)
"Thirteen patients with Wegener's granulomatosis were seen over 10."	1.27	Wegener's granulomatosis. Clinical features and outcome in 13 patients. ( Brandwein, S; Danoff, D; Esdaile, J; Tannenbaum, H, 1983)
"A case of Wegener's granulomatosis is reported which manifested episcleritis followed by scleritis, and followed in turn by separate episodes of right and left orbital pseudotumor spanning 15 years prior to respiratory tract disease."	1.27	Chronic ophthalmic Wegener's granulomatosis. ( Coppeto, JR; Monteiro, ML; Yamase, H, 1985)
"Two cases of Wegener's granulomatosis are reported."	1.27	[Spontaneous course of Wegener's granulomatosis]. ( Adam, O; Wiesinger, H, 1988)
"A patient with Wegener's granulomatosis sustained a myocardial infarction."	1.27	Reversible valvulitis in Wegener's granulomatosis. ( Gerbracht, DD; Savage, RW; Scharff, N, 1987)
"We describe a patient with Wegener's granulomatosis who presented with erosive arthritis."	1.27	Wegener's granulomatosis presenting with erosive arthritis. ( Brower, A; Jacobs, RP; Moore, M, 1987)
"The authors report a case of Wegener's granulomatosis, whose initial manifestation was an isolated endobronchial stenosis."	1.27	[Bronchial involvement disclosing Wegener's granulomatosis]. ( Breton, JL; Depierre, A; Dubiez, A; Garnier, G; Ranfaing, E, 1986)
"Overlooking the literature, Wegener's granulomatosis often seems to have manifestations in the ear-nose-throat region, especially in the beginning."	1.27	[Wegener's granulomatosis in the ORL area]. ( Ampe, W; D'Hont, G; Depondt, M; Malfroid, F, 1986)
"Twelve patients with Wegener's granulomatosis were treated with antimicrobial agents, chiefly trimethoprim-sulfamethoxazole."	1.27	Wegener's granulomatosis: observations on treatment with antimicrobial agents. ( DeRemee, RA; McDonald, TJ; Weiland, LH, 1985)
"Patient 1 has been free of recurrence for 11 years, six of them without treatment."	1.27	[Long-term observations of Wegener's disease. Results of treating recurrences]. ( Bohn, T; Ho, AD; Hunstein, W, 1985)
"We report four children with Wegener granulomatosis in whom the initial clinical findings suggested Henoch-Schönlein purpura."	1.27	Wegener granulomatosis in pediatric patients. ( Beatty, EC; Duggan, E; Hall, SL; Hellerstein, S; Mauer, SM; Miller, LC, 1985)
"Nine patients with Wegener's granulomatosis have developed chronic renal failure and undergone chronic hemodialysis and transplantation."	1.26	Wegener's granulomatosis with severe renal failure: clinical course and results of dialysis and transplantation. ( Davin, T; Kjellstrand, CM; Kuross, S, 1981)
"The patient also developed aortic regurgitation, a cardiac lesion not previously described in this disorder."	1.26	Prolonged anuria and aortic insufficiency in a child with Wegener's granulomatosis. ( Chevalier, RL; Dabbagh, S; Sturgill, BC, 1982)
"Exudative retinal detachment is a finding not previously described clinically, but alluded to in pathologic descriptions from case reports."	1.26	Exudative retinal detachment in Wegener's granulomatosis: case report. ( Jaben, SL; Norton, EW, 1982)
"Both patients suffered lasting hearing impairment in spite of general improvement during the course of the disease."	1.26	Hearing impairment and facial palsy as initial signs of Wegener's granulomatosis. ( Calonius, IH; Christensen, CK, 1980)
"In a casuistic report of a case of Wegener's granulomatosis the diagnostic and therapeutic problems of a relatively rare clinical picture are described."	1.26	[The unusual case: Wegener's granulomatosis]. ( Fünfstück, R; Linde, M; Schulze, E; Sperschneider, H; Stein, G; Wessel, G, 1980)
"However, intermittent atelectasis and pneumonia occurred distal to a residual short stricture."	1.26	Radiation therapy and mechanical dilation of endobronchial obstruction secondary to Wegener's granulomatosis. ( Bilinsky, RT; Eagleton, LE; Hawe, A; Rosher, RB, 1979)
" Haemodialysis was started, cytotoxic drugs were stopped, and steroid dosage was reduced."	1.26	Haemodialysis and transplantation in Wegener's granulomatosis. ( Alexandre, GP; Pirson, Y; van Ypersele de Strihou, C; Vandenbroucke, JM, 1979)
"We report a case of Wegener granulomatosis presenting as a destructive urethral mass."	1.26	Wegener granulomatosis. Unusual cause of necrotizing urethritis. ( Bowles, WT; Fowler, M; Katzenstein, AL; Martin, SA; Packman, R, 1979)
"Subacute meningitis was the first manifestation of illness in a young man with multisystem disease involving lung, kidney, eye, and central nervous system."	1.26	Subacute meningitis heralding a diffuse granulomatous angiitis: (Wegener's granulomatosis?). ( Atcheson, SG; Van Horn, G, 1977)
"Over a 16 year period, 47 patients with Wegener's granulomatosis were treated at the National Institutes of Health."	1.26	Diagnostic and therapeutic aspects of the surgical approach to Wegener's granulomatosis. ( Fauci, AS; Flye, MW; Mundinger, GH, 1979)
"Two patients with Wegener's granulomatosis, who were in complete remission secondary to cyclophosphamide therapy but who had end-stage renal failure, were treated with renal transplantation."	1.26	Successful renal transplantation in Wegener's granulomatosis. ( Balow, JE; Brown, R; Chazan, J; Fauci, AS; Monoaco, AP; Sahyoun, AI; Steinman, T; Wolff, SM, 1976)
"On the basis of 5 casuistics of Wegener's granulomatosis the author deals with the difficulties of the diagnosis and differential diagnosis, whereby above all is referred to the preculiarities of the cases and the malinterpretation of the diagnoses on admission to the hospital."	1.26	[Contribution to the clinical picture of Wegener's granulomatosis]. ( Ehrig, J; Lehmann, K; Rössner, B; Rübesamen, M; Wessel, G, 1976)
"Thirteen patients with Wegener's granulomatosis have been treated with cytotoxic agents."	1.25	Treatment of Wegener's granulomatosis of lung. ( Isreal, HL; Patchfsky, AS, 1975)
"Bilateral uveitis was the initial manifestation of a limited form of Wegener granulomatosis in a 47-year-old man."	1.25	Limited form of Wegener granulomatosis. Eye involvement as a major sign. ( Coutu, RE; Friedman, E; Klein, M; Lessell, S; Snider, GL, 1975)

Research

Studies (349)

Authors

Clinical Trials (25)

Trial Overview

Trial Outcomes

Number of Subjects Experiencing a Relapse After Previously Achieving BVAS=0 During the Study

Timeframe	Studies, this research(%)	All Research%
pre-1990	129 (36.96)	18.7374
1990's	78 (22.35)	18.2507
2000's	71 (20.34)	29.6817
2010's	55 (15.76)	24.3611
2020's	16 (4.58)	2.80

Authors	Studies
Donaldson, L	1
Pagnoux, C	6
Margolin, E	1
Harigai, M	2
Takada, H	1
Kaname, S	1
Tamura, N	2
Dobashi, H	1
Kubono, S	1
Yoshida, T	1
Makiya, MA	1
Khoury, P	1
Kuang, FL	1
Mata, AD	1
Mahmood, S	1
Bowman, A	1
Espinoza, D	1
Kovacs, N	1
Brown, T	1
Holland, N	1
Wetzler, L	1
Ware, JM	1
Dyer, AM	1
Akuthota, P	1
Bochner, BS	1
Chinchilli, VM	1
Gleich, GJ	1
Langford, C	1
Merkel, PA	6
Specks, U	7
Weller, PF	1
Wechsler, ME	3
Prussin, C	1
Fay, MP	1
Klion, AD	1
Özdel Öztürk, B	1
Yavuz, Z	1
Aydın, Ö	1
Mungan, D	1
Sin, BA	1
Demirel, YS	1
Bavbek, S	1
Frątczak, A	1
Polak, K	1
Miziołek, B	1
Bergler-Czop, B	1
Chen, Y	1
Wan, Q	1
Liu, B	1
Cottu, A	1
Groh, M	2
Desaintjean, C	1
Marchand-Adam, S	2
Guillevin, L	7
Puechal, X	4
Beaumesnil, S	1
Lazaro, E	1
Samson, M	2
Taille, C	2
Durel, CA	2
Diot, E	2
Nicolas, S	1
Guilleminault, L	1
Ebbo, M	1
Cathebras, P	1
Dupin, C	1
Yildiz, H	1
Belfeki, N	1
Pugnet, G	2
Chauvin, P	1
Jouneau, S	1
Lifermann, F	2
Martellosio, JP	1
Cottin, V	2
Terrier, B	3
Molina, B	1
Padoan, R	1
Urban, ML	1
Novikov, P	1
Caminati, M	1
Néel, A	1
Bouillet, L	1
Fraticelli, P	1
Schleinitz, N	1
Christides, C	1
Moi, L	1
Godeau, B	2
Knight, A	1
Schroeder, JW	1
Gil, H	1
Gelain, E	1
Lerais, B	1
Ruivard, M	2
Moroni, L	1
Thiel, J	1
Kernder, A	1
Cohen Tervaert, JW	1
Costanzo, G	1
Folci, M	1
Rizzello, S	1
Cohen, P	4
Emmi, G	1
Riancho-Zarrabeitia, L	1
Peiró Callizo, E	1
Drake-Pérez, M	1
García Montesinos, B	1
Terán, N	1
Martínez-Taboada, VM	1
Starr, MR	1
Garrity, JA	1
Tooley, AA	1
Salomão, DR	1
Piccirilli, G	1
Chiereghin, A	1
Maritati, M	1
Turello, G	1
Felici, S	1
La Corte, R	2
Gabrielli, L	1
Contini, C	1
Lazzarotto, T	1
Vergles, M	1
Matković, Z	1
Lalić, K	1
Trkanjec, JT	1
Tudorić, N	1
Boncoraglio, MT	1
Prieto-González, S	1
Fernandes-Serodio, J	1
Corral-Molina, JM	1
Solé, M	1
Hernández-Rodríguez, J	1
Manka, LA	1
Guntur, VP	1
Denson, JL	1
Dunn, RM	1
Dollin, YT	1
Strand, MJ	1
Beijer, E	1
Roodenburg-Benschop, C	1
Schimmelpennink, MC	1
Grutters, JC	1
Meek, B	1
Veltkamp, M	1
Frey, J	1
Kramer, J	1
Castellani, R	1
Sriwastava, S	1
Divakaran, S	2
Vaidya, A	1
Kobzik, L	2
Dellaripa, P	2
Levy, B	1
Loscalzo, J	1
Perez, MO	1
Oliveira, RM	1
Levy-Neto, M	1
Caparbo, VF	1
Pereira, RMR	1
Joseph, MX	1
Jenkins, LE	1
Davis, LS	1
Mongera, N	1
Di Maso, V	1
Ermacora, E	1
Carraro, M	1
Bregant, C	1
Pian, M	1
Savi, U	1
Lorenzon, E	1
Boscutti, G	1
Shaukat, MS	1
Ocon, AJ	1
Peredo, RA	1
Bhatt, BD	1
Laurent, C	1
Dion, J	1
Régent, A	1
Parperis, K	1
Abdulqader, Y	1
Chasseur, P	1
Blockmans, D	1
von Frenckell, C	1
Nicolas, JB	1
Regniers, C	1
Vandergheynst, F	1
Kocaer, SB	1
Ozer, E	1
Yilmaz, E	1
Sari, I	1
Ramirez, GA	1
Rovere-Querini, P	1
Blasi, M	1
Sartorelli, S	1
Di Chio, MC	1
Baldini, M	1
De Lorenzo, R	1
Bozzolo, EP	1
Leone, R	1
Mantovani, A	1
Manfredi, AA	1
Tombetti, E	1
Lee, JJY	1
Alsaleem, A	1
Chiang, GPK	1
Limenis, E	1
Sontichai, W	1
Yeung, RSM	1
Akikusa, J	1
Laxer, RM	2
Kenis, I	1
Zahavi, T	1
Korzets, Z	1
Perez Alamino, R	1
Espinoza, LR	2
Sethi, S	1
Machuzak, MS	1
Sosna, J	1
Malm, IJ	1
Mener, DJ	1
Kim, J	1
Seo, P	4
Kim, YJ	1
Aldasoro-Cáceres, V	1
Aldasoro-Cáceres, I	1
Pérez-Moreiras, JV	1
Murié-Fernández, M	1
Ibáñez-Bosch, R	1
Langford, CA	8
Monach, PA	3
Cuthbertson, D	1
McAlear, CA	2
Ytterberg, SR	3
Hoffman, GS	11
Krischer, JP	1
Lowenthal, MN	1
Chorny, I	1
Tishchenko, L	1
West, S	1
Kenedi, C	1
Chen, AY	1
Al-Fakhouri, A	1
Manadan, A	1
Gan, J	1
Sreih, AG	2
Calich, AL	1
London, J	1
Charles, P	1
Mouthon, L	4
Bernauer, W	1
Pleisch, B	1
Brunner, M	1
Parlow, B	1
Mohiuddin, A	1
Chou, T	1
Cato-Varlack, J	1
Boneparth, A	1
Moorthy, LN	1
Chang, CY	1
Marzan, KA	1
O'Neil, L	1
Robinson, D	1
Hamza, S	1
El-Gabalawy, H	1
Liu, X	1
Kuriyan, AE	1
Amescua, G	1
Sakaue, S	1
Sumitomo, S	1
Fujio, K	1
Yamamoto, K	1
Flores-Suárez, LF	1
Saldarriaga Rivera, LM	1
Rivera Rosales, RM	1
Ruiz, N	1
Báez Saldaña, R	1
Miloslavsky, EM	1
Spiera, R	3
Kallenberg, CG	3
St Clair, EW	3
Tchao, NK	2
Ding, L	2
Iklé, D	2
Villareal, M	1
Lim, N	1
Brunetta, P	2
Fervenza, FC	3
Stone, JH	8
Böhm, M	1
Basson, S	1
Pajor, AM	1
Kwiatkowska, S	1
Kroczyńska-Bednarek, J	1
Piotrowski, WJ	1
Kohn, R	1
Margolis, ML	1
Kaushik, P	1
Anderson, JT	1
Dugar, S	1
Mathew, R	1
Nikolic, B	1
Patel, R	1
Detoraki, A	1
Di Capua, L	1
Varricchi, G	1
Genovese, A	1
Marone, G	1
Spadaro, G	1
Aragonès, JM	1
Arias-Rivero, M	1
García-Barrionuevo, JM	1
Lucchetti, G	1
Kindle, SA	1
Camilleri, MJ	1
Gibson, LE	1
Davis, MD	1
Fukui, S	1
Iwamoto, N	1
Mochizuki, Y	1
Nakajima, H	1
Abe, K	1
Kawakami, A	1
Varnier, GC	1
Sebire, N	1
Christov, G	1
Eleftheriou, D	1
Brogan, PA	1
Faurschou, M	1
Ahlström, MG	1
Lindhardsen, J	1
Obel, N	1
Baslund, B	1
Krischer, J	1
Cronholm, PF	1
Burroughs, C	1
Borchin, R	1
Easley, E	1
Davis, T	1
Kullman, J	1
Carette, S	1
Khalidi, N	1
Koening, C	1
Monach, P	1
Moreland, L	1
Ytterberg, S	1
Cossack, M	1
Vanam, S	1
Wallace, B	1
Brass, SD	1
Durand, ML	1
Chen, JW	1
Stone, JR	1
Khellaf, M	2
Mahr, A	3
Hamidou, MA	1
Boffa, JJ	1
Ducroix, JP	1
Kyndt, X	1
Papo, T	3
Lambert, M	1
Le Noach, J	1
Merrien, D	1
Vinzio, S	2
Cordier, JF	3
Xue, J	1
Wang, H	1
Wu, H	1
Jin, Q	1
Koch, M	1
Kohnle, M	1
Trapp, R	1
Cantoni, N	1
Meyer, P	1
Katan, M	1
Kappos, L	1
Hess, C	1
Daikeler, T	1
Purvin, V	1
Kawasaki, A	1
Ketata, W	1
Msaad, S	1
Gharsalli, H	1
Kammoun, K	1
Abid, T	1
Mrabet, S	1
Makni, S	1
Boudawara, T	1
Ayoub, A	1
Huerva, V	1
Sanchez, MC	1
Traveset, A	1
Jurjo, C	1
Ruiz, A	1
Marie, I	2
Lahaxe, L	1
Josse, S	1
Lequerre, T	1
Vittecoq, O	1
Savoye, G	1
Turkiewicz, A	1
Webber, L	1
Sejismundo, LP	1
Mieras, K	1
Weitzenkamp, D	1
Seyfert-Margolis, V	1
Mueller, M	1
Allen, NB	2
Geetha, D	1
Keogh, KA	2
Kissin, EY	1
Peikert, T	1
Stegeman, C	1
Sánchez-Escuredo, A	1
Núñez, R	1
Ibernón, M	1
Martínez, E	1
López, D	1
Navarro, M	1
Bonet, J	1
Ara, J	1
Romero, R	1
Kandiah, DA	1
Müller, S	1
Eljack, S	1
DelGaudio, JM	1
Dufour, JF	1
Le Gallou, T	1
Aumaître, O	1
Pinède, L	1
Aslangul, E	1
Decaux, O	1
Dubois, A	2
Agard, C	1
Comoz, F	1
Boutemy, J	1
Broussolle, C	1
Sève, P	1
Bienvenu, B	1
Kahraman, H	1
Inci, MF	1
Tokur, M	1
Cetin, GY	1
Ghez, D	1
Westeel, PF	1
Henry, I	1
Pruna, A	1
Fournier, A	1
Lassoued, K	1
Huang, PT	1
Bhamra, J	1
Huang, JT	1
Talar-Williams, C	2
Barron, KS	1
Sneller, MC	4
Kuchel, J	1
Lee, S	1
Sinovich, V	1
Snow, J	1
PausJenssen, ES	1
Cockcroft, DW	1
Korzeniewska-Koseła, M	1
Maziarka, D	1
Sobiecka, M	1
Bestry, I	1
Langfort, R	1
Demkow, U	1
Kuś, J	1
Fam, AG	1
Lavine, E	1
Lee, L	1
Perez-Ordonez, B	1
Goyal, M	1
BEIDLEMAN, B	1
CLARKE, PB	1
SILTZBACH, LE	1
PANZRAM, G	1
MEERBACH, W	1
RUEBESAMEN, M	1
HUBER, J	1
Klammer, M	1
Hetzel, GR	1
Fürst, G	1
Gabbert, HE	1
Megahed, M	1
Ruzicka, T	1
Hengge, UR	1
Bleeker-Rovers, CP	1
Bredie, SJ	1
van der Meer, JW	1
Corstens, FH	1
Oyen, WJ	1
Casanova, A	1
Pérez Amor, E	1
Girón, RM	1
Zamora, E	1
Vélez, MD	1
Ancochea, J	1
Kolyvanos Naumann, U	2
Käser, L	2
Vetter, W	2

Trial	Phase	Enrollment	Study Type	Start Date	Status
A Randomized, Double-Blind, Active-Controlled, Phase 3 Study to Evaluate the Safety and Efficacy of CCX168 (Avacopan) in Patients With ANCA-Associated Vasculitis Treated Concomitantly With Rituximab or Cyclophosphamide/Azathioprine[NCT02994927]	Phase 3	331 participants (Actual)	Interventional	2017-03-15	Completed
Open-Label, to Evaluate the Efficacy and Safety of Reslizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) Study: RITE Study[NCT02947945]	Phase 2	10 participants (Anticipated)	Interventional	2017-09-12	Recruiting
Salvage Therapy for Patients With Inadequate Response to Standard of Care Therapy in Granulomatosis With Polyangiitis[NCT04871191]	Phase 2	42 participants (Anticipated)	Interventional	2023-03-31	Not yet recruiting
A Multi-Center, Open-label Pilot Study of Abatacept (CTLA4-Ig) in the Treatment of Mild Relapsing Wegener's Granulomatosis[NCT00468208]	Phase 1/Phase 2	20 participants (Actual)	Interventional	2008-02-29	Completed
Rituximab Therapy for the Induction of Remission and Tolerance in ANCA-Associated Vasculitis (ITN021AI)[NCT00104299]	Phase 2/Phase 3	197 participants (Actual)	Interventional	2005-01-31	Completed
The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach[NCT01933724]	Phase 3	12 participants (Actual)	Interventional	2014-02-17	Active, not recruiting
Treatment of ANCA-Associated Vasculitides : Corticosteroids and Pulse Cyclophosphamide Followed by Maintenance Therapy With Methotrexate or Azathioprine: a Prospective Multicenter Randomized Trial[NCT00349674]	Phase 3	126 participants	Interventional	1999-01-31	Active, not recruiting
Rituximab for the Otolaryngologic Manifestations of Granulomatosis With Polyangiitis[NCT02626845]	Phase 4	3 participants (Actual)	Interventional	2015-12-31	Terminated (stopped due to Slow recruitment)
Low-dose Glucocorticoids Plus Rituximab Versus High-dose Glucocorticoids Plus Rituximab for Remission Induction in ANCA-associated Vasculitis; a Multicentre, Open Label, Randomised Control Trial[NCT02198248]	Phase 4	140 participants (Actual)	Interventional	2014-10-31	Active, not recruiting
Biosimilars of Rituximab in ANCA-associated Vasculitis Compared to the Originator (BRAVO): a CanVasc Multicentre Study[NCT05716334]		240 participants (Anticipated)	Observational	2021-06-15	Recruiting
Rituximab for Anti-neutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis (RAVE) Long-Term Follow-Up Study[NCT01586858]		67 participants (Actual)	Observational	2012-05-31	Terminated (stopped due to Loss of Funding)
Maintenance of ANCA Vasculitis Remission by Intermittent Rituximab Dosing Based on B-cell Reconstitution vs a Serologic ANCA Flare[NCT02749292]	Phase 4	115 participants (Actual)	Interventional	2016-06-30	Terminated (stopped due to Due to the coronavirus disease 2019 (COVID-19) pandemic and the deleterious impact of rituximab on vaccination efficacy, the trial was concluded before reaching the target enrollment of 200.)
Efficacy and Safety of Rituximab in the Treatment of Good Prognosis Microscopic Polyangiitis[NCT03920722]	Phase 3	8 participants (Actual)	Interventional	2020-10-24	Active, not recruiting
An International, Open Label, Randomised Controlled Trial Comparing Rituximab With Azathioprine as Maintenance Therapy in Relapsing ANCA-associated Vasculitis[NCT01697267]	Phase 3	188 participants (Actual)	Interventional	2013-04-30	Completed
Intravenous Immunoglobulin After Relapse in Vasculitis (Microscopic Polyangiitis, Wegener's Granulomatosis and SHURG-STRAUSS Syndrome) During and After Corticosteroids and Immunosuppressant Therapies a Multicenter Prospective Trial[NCT00307658]	Phase 3	40 participants	Interventional	2001-03-31	Terminated
Phase I/II Trial of TNFR:Fc (Etanercept) in Patients With Wegener's Granulomatosis[NCT00001901]	Phase 2	60 participants	Interventional	1999-02-28	Completed
A Staged Therapeutic Approach Using Cyclophosphamide and Methotrexate in the Treatment of Wegener's Granulomatosis and Related Vasculitides[NCT00001473]		100 participants	Observational	1995-03-31	Completed
An Open Trial of the Efficacy of Glucocorticoids and Methotrexate (MTX) in the Treatment of Systemic Vasculitis[NCT00001256]	Phase 2	100 participants	Interventional	1990-03-31	Completed
A Randomized Trial Examining the Use of Daclizumab in Wegener's Granulomatosis[NCT00040248]	Phase 2	75 participants	Interventional	2002-06-30	Completed
An Open Label Pilot Study Examining the Use of Rituximab in Patients With Wegener's Granulomatosis Who Have Experienced Disease Relapse on Standard Therapies[NCT00072592]	Phase 1	10 participants	Interventional	2003-10-31	Completed
Phase I/II Gene Transfer Clinical Trial of rAAV9.CMV.hNAGLU for Mucopolysaccharidosis (MPS) IIIB[NCT03315182]	Phase 1/Phase 2	11 participants (Actual)	Interventional	2017-10-16	Terminated (stopped due to Abeona has decided to discontinue development activities for Product ABO-101 due to a lack of drug supply and for business reasons unrelated to the product safety profile and/or signs of efficacy)
Phase I Trial of Recombinant Human Interleukin-10 (SCH 52000) in Patients With Wegener's Granulomatosis[NCT00001761]	Phase 1	15 participants	Interventional	1998-02-28	Completed
Treatment of Wegener's Granulomatosis With Cyclophosphamide[NCT00001155]	Phase 2	200 participants	Interventional	1976-02-29	Completed
Cyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides. A Randomized Controlled Trial.[NCT00751517]	Phase 2	0 participants	Interventional		Active, not recruiting
A Randomized Trial Comparing Methotrexate Versus Mycophenolate Mofetil for Remission Maintenance in Wegener's Granulomatosis and Related Vasculitides[NCT00004567]	Phase 2	75 participants	Interventional	2000-02-29	Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

"BVAS=Birmingham Vasculitis Activity Score;~A relapse was defined as occurrence of at least one major item in the BVAS, or three or more minor items in the BVAS, or one or two minor items in the BVAS recorded at two consecutive visits, after:~having achieved remission at Week 26 (BVAS=0 and no glucocorticoids for ANCA-associated vasculitis within 4 weeks) or~having achieved BVAS=0 at any time during the treatment period~The BVAS form is divided into 9 organ-based systems, with each section including symptoms/signs that are typical of that particular organ involvement in systemic vasculitis. The clinician only scores features believed to be due to active vasculitis. Completion of the form provides a numerical score, which ranges from 0 (best health) to 63 (worst health)." (NCT02994927)
Timeframe: From day 1 throughout the study period (day 421/week 60)

Number of Subjects With Clinically Significant ECG Changes From Baseline

Intervention	Participants (Count of Participants)
Prednisone Group	33
Avacopan Group	16

"Clinical significance was assessed by the individual reading of the ECGs~ECG=Electrocardiogram" (NCT02994927)
Timeframe: From day 1 throughout the study period (day 421/week 60)

Percentage of Participants With BVAS of 0 at Week 4, Regardless of Whether the Subjects Received Glucocorticoids During This Period of Time and Based on Assessment by the Blinded AC

Intervention	Participants (Count of Participants)
Prednisone Group	8
Avacopan Group	12

"AC=Adjudication Committee; BVAS=Birmingham Vasculitis Activity Score;~The BVAS form is divided into 9 organ-based systems, with each section including symptoms/signs that are typical of that particular organ involvement in systemic vasculitis. The clinician only scores features believed to be due to active vasculitis. Completion of the form provides a numerical score, which ranges from 0 (best health) to 63 (worst health)." (NCT02994927)
Timeframe: Week 4

Percentage of Subjects Achieving Disease Remission at Week 26

Intervention	percentage of participants (Number)
Prednisone Group	68.9
Avacopan Group	62.7

"Disease remission at Week 26 was defined as:~Achieving a BVAS of 0 as determined by the Adjudication Committee;~No administration of glucocorticoids given for ANCA-associated vasculitis within 4 weeks prior to Week 26;~No BVAS >0 during the 4 weeks prior to Week 26 (if collected for an unscheduled assessment)." (NCT02994927)
Timeframe: Week 26

Percentage of Subjects Achieving Sustained Disease Remission at Week 52

Intervention	percentage of participants (Number)
Prednisone Group	70.1
Avacopan Group	72.3

"Sustained remission at Week 52 was defined as:~Disease remission at Week 26 as defined above;~Disease remission at Week 52 defined as a BVAS of 0 at Week 52 as determined by the Adjudication Committee and no administration of glucocorticoids for treatment of ANCA-associated vasculitis within 4 weeks prior to Week 52;~No disease relapse between Week 26 and Week 52 as determined by the Adjudication Committee." (NCT02994927)
Timeframe: Week 52

Percentage of Subjects and Time to Experiencing a Relapse After Previously Achieving BVAS=0 at Any Time During the Treatment Period

Intervention	percentage of participants (Number)
Prednisone Group	54.9
Avacopan Group	65.7

"The median time to relapse was not estimable because of small number of relapsed subjects.~A relapse was defined as occurrence of at least one major item in the BVAS, or three or more minor items in the BVAS, or one or two minor items in the BVAS recorded at two consecutive visits, after:~having achieved remission at Week 26 (BVAS=0 and no glucocorticoids for ANCA-associated vasculitis within 4 weeks) or~having achieved BVAS=0 at any time during the treatment period~The Birmingham Vasculitis Activity Score (BVAS) form is divided into 9 organ-based systems, with each section including symptoms/signs that are typical of that particular organ involvement in systemic vasculitis. The clinician only scores features believed to be due to active vasculitis. Completion of the form provides a numerical score, which ranges from 0 (best health) to 63 (worst health)." (NCT02994927)
Timeframe: Week 52

Percentage of Subjects and Time to Experiencing a Relapse After Previously Achieving Remission at Week 26 in the Study

Intervention	percentage of participants (Number)
Prednisone Group	21.0
Avacopan Group	10.1

"The median time to relapse was not estimable because of small number of relapsed subjects.~A relapse was defined as occurrence of at least one major item in the BVAS, or three or more minor items in the BVAS, or one or two minor items in the BVAS recorded at two consecutive visits, after:~having achieved remission at Week 26 (BVAS=0 and no glucocorticoids for ANCA-associated vasculitis within 4 weeks) or~having achieved BVAS=0 at any time during the treatment period~ANCA=anti-neutrophil cytoplasmic autoantibody; BVAS=Birmingham Vasculitis Activity Score; The BVAS form is divided into 9 organ-based systems, with each section including symptoms/signs that are typical of that particular organ involvement in systemic vasculitis. The clinician only scores features believed to be due to active vasculitis. Completion of the form provides a numerical score, which ranges from 0 (best health) to 63 (worst health)." (NCT02994927)
Timeframe: Week 52

Certain Safety Endpoints of Interest: Infections, Hepatic System Abnormalities, WBC Count Decreases, and Hypersensitivity

Intervention	percentage of participants (Number)
Prednisone Group	12.2
Avacopan Group	7.5

"WBC=White Blood Cell~TEAE=Treatment-Emergent Adverse Event" (NCT02994927)
Timeframe: From day 1 throughout the study period (day 421/week 60)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (1/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (2/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (3/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (4/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (5/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Serum Chemistry (1/2)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Serum Chemistry (2/2)

Change From Baseline in Vital Signs (1/5)

Change From Baseline in Vital Signs (2/5)

Change From Baseline in Vital Signs (3/5)

Change From Baseline in Vital Signs (4/5)

Change From Baseline in Vital Signs (5/5)

Change From Baseline Over 52 Weeks in Health-related Quality of Life as Measured by the Domains and Component Scores of the SF-36v2 and EQ-5D-5L VAS and Index

Intervention	Participants (Count of Participants)
	Any Treatment-Emergent Infection	Any Serious Treatment-Emergent Infection	Any Severe Treatment-Emergent Infection	Any Treatment-Emergent Infection Leading to Study Withdrawal	Any Treatment-Emergent Life-threatening Infection	Any Treatment-Emergent Infection Leading to Death	Any TEAE Associated with Hepatic Abnormalities	Any TEAE Associated with Low WBC Counts	Any TEAE Associated with hypersensitivity
Avacopan Group	113	22	12	4	1	1	22	31	68
Prednisone Group	124	25	10	5	2	2	19	39	70

Intervention	10^3 cells/μL (Mean)
	Leukocytes (Week 26)	Leukocytes (Week 52)	Neutrophils (Week 26)	Neutrophils (Week 52)	Lymphocytes (Week 26)	Lymphocytes (Week 52)
Avacopan Group	-5.94	-5.62	-5.24	-4.95	-0.84	-0.82
Prednisone Group	-5.69	-5.54	-5.10	-4.89	-0.62	-0.67

Intervention	10^9 cells/L (Mean)
	Eosinophils (Week 26)	Eosinophils (Week 52)	Basophils (Week 26)	Basophils (Week 52)	Monocytes (Week 26)	Monocytes (Week 52)	Platelets (Week 26)	Platelets (Week 52)
Avacopan Group	0.07	0.07	-0.00	-0.01	-0.04	-0.01	-77.1	-73.8
Prednisone Group	0.07	0.05	-0.01	-0.01	0.01	0.01	-73.9	-75.5

Intervention	10^12 cells/L (Mean)
	Erythrocytes (Week 26)	Erythrocytes (Week 52)
Avacopan Group	0.252	0.279
Prednisone Group	0.226	0.244

Intervention	g/dL (Mean)
	Hemoglobin (Week 26)	Hemoglobin (Week 52)
Avacopan Group	1.10	1.27
Prednisone Group	1.07	1.20

Intervention	percentage of red blood cells (Mean)
	Hematocrit (Week 26)	Hematocrit (Week 52)
Avacopan Group	2.7	3.2
Prednisone Group	2.6	3.0

Intervention	U/L (Mean)
	Lactate Dehydrogenase (Week 26)	Lactate Dehydrogenase (Week 52)	Alkaline Phosphatase (Week 26)	Alkaline Phosphatase (Week 52)	Creatine Kinase (Week 26)	Creatine Kinase (Week 52)	Alanine Aminotransferase (Week 26)	Alanine Aminotransferase (Week 52)	Aspartate Aminotransferase (Week 26)	Aspartate Aminotransferase (Week 52)
Avacopan Group	-6.1	-10.7	-3.9	-4.0	76.8	76.3	-6.1	-7.2	2.5	2.0
Prednisone Group	2.3	-8.6	-0.6	0.8	47.6	57.6	-6.8	-8.2	1.9	0.5

Intervention	mg/dL (Mean)
	Creatinine (Week 26)	Creatinine (Week 52)	Urea Nitrogen (Week 26)	Urea Nitrogen (Week 52)	Protein (Week 26)	Protein (Week 52)	Cholesterol (Week 26)	Cholesterol (Week 52)	LDL Cholesterol (Week 26)	LDL Cholesterol (Week 52)	Bilirubin (Week 26)	Bilirubin (Week 52)
Avacopan Group	-0.195	-0.244	-11.0	-11.9	220	250	7.4	9.3	12.0	11.9	0.078	0.057
Prednisone Group	-0.105	-0.200	-9.4	-7.8	50	160	19.0	13.8	22.7	21.7	0.065	0.053

Intervention	mmHg (Mean)
	Systolic Blood Pressure (Week 26)	Systolic Blood Pressure (Week 52)	Diastolic Blood Pressure (Week 26)	Diastolic Blood Pressure (Week 52)
Avacopan Group	-2.6	-1.0	0.5	1.4
Prednisone Group	-2.5	-2.4	2.7	1.4

Intervention	beats/min (Mean)
	Pulse Rate (Week 26)	Pulse Rate (Week 52)
Avacopan Group	0.9	-0.3
Prednisone Group	2.2	-1.3

Intervention	degree Celsius (Mean)
	Temperature (Week 26)	Temperature (Week 52)
Avacopan Group	-0.11	-0.11
Prednisone Group	-0.03	0.04

Intervention	kilogram(s) (Mean)
	Weight (Week 26)	Weight (Week 52)
Avacopan Group	1.93	2.59
Prednisone Group	3.33	3.27

Intervention	kilogram(s)/ square meter (Mean)
	BMI (Week 26)	BMI (Week 52)
Avacopan Group	0.67	0.94
Prednisone Group	1.13	1.12

"SF-36v2: Measure of health- related quality of life (Medical Outcomes Survey Short Form-36 version 2)~EQ-5D-5L: EuroQuality of Life-5 Domains-5 Levels~The SF-36v2 component scores and the EQ-5D-5L VAS score range from 0 (worst health) to 100 (best health). The EQ-5D-5L Index Score ranges from 0 (worst health) to 1 (best health)." (NCT02994927)
Timeframe: Baseline, Week 26 and 52

Change in the VDI From Baseline Over 52 Weeks, Including the Week 26 and Week 52 Time Points

Intervention	Change from baseline (Least Squares Mean)
	SF-36v2: Physical Component Score (Week 26)	SF-36v2: Physical Component Score (Week 52)	SF-36v2: Physical Functioning (Week 26)	SF-36v2: Physical Functioning (Week 52)	SF-36v2: Role Physical (Week 26)	SF-36v2: Role Physical (Week 52)	SF-36v2: Bodily Pain (Week 26)	SF-36v2: Bodily Pain (Week 52)	SF-36v2: General Health Perception (Week 26)	SF-36v2: General Health Perception (Week 52)	SF-36v2: Mental Component Score (Week 26)	SF-36v2: Mental Component Score (Week 52)	SF-36v2: Mental Health (Week 26)	SF-36v2: Mental Health (Week 52)	SF-36v2: Role Emotional (Week 26)	SF-36v2: Role Emotional (Week 52)	SF-36v2: Social Functioning (Week 26)	SF-36v2: Social Functioning (Week 52)	SF-36v2: Vitality (Week 26)	SF-36v2: Vitality (Week 52)	EQ-5D-5L VAS Score (Week 26)	EQ-5D-5L VAS Score (Week 52)	EQ-5D-5L Index Score (Week 26)	EQ-5D-5L Index Score (Week 52)
Avacopan Group	4.445	4.980	7.31	9.55	16.78	17.12	14.75	16.12	3.12	5.84	4.849	6.394	8.29	10.89	7.32	9.38	14.50	18.06	12.03	14.36	9.1	13.0	0.0229	0.0474
Prednisone Group	1.344	2.626	1.88	4.82	7.52	12.27	9.82	11.87	-2.89	-0.17	3.271	4.694	6.84	9.66	1.40	4.14	11.09	13.56	6.42	10.48	5.5	7.1	-0.0010	-0.0038

VDI=Vasculitis Damage Index; The VDI is comprised of 64 items of damage, grouped into 11 organ-based systems or categorizations. Damage is defined as the presence of non-healing scars and does not give any indication of current disease activity. Damage is also defined as having been present or currently present for at least 3 months. Completion of the form provides a numerical score, which ranges from 0 (best health) to 64 (worst health). (NCT02994927)
Timeframe: Baseline, Week 26 and 52

Glucocorticoid-induced Toxicity as Measured by Change From Baseline Over the First 26 Weeks in the GTI

Intervention	score on a scale (Least Squares Mean)
	Week 26	Week 52
Avacopan Group	1.06	1.17
Prednisone Group	0.97	1.15

"GTI-CWS=Glucocorticoid Toxicity Index Cumulative Worsening Score;~GTI-AIS=Glucocorticoid Toxicity Index Aggregate Improvement Score;~The Glucocorticoid Toxicity Index (GTI) was developed to score glucocorticoid toxicity. The GTI includes: the Cumulative Worsening Score (CWS) that captures cumulative toxicity, both permanent and transient, over the course of time (serves as a cumulative record of toxicity); and the Aggregate Improvement Score that captures both improvement and worsening of toxicity over time (serves as a record of both improving and worsening toxicity). Both scores range from 0 (best health) to 100 (worst health)." (NCT02994927)
Timeframe: Baseline, Week 13 and 26

In Subjects With Renal Disease and Albuminuria at Baseline (Based in the BVAS Renal Component), the Percent Change in UACR From Baseline Over 52 Weeks

Intervention	Glucocorticoid Toxicity Index (Least Squares Mean)
	GTI-CWS (Week 13)	GTI-CWS (Week 26)	GTI-AIS (Week 13)	GTI-AIS (Week 26)
Avacopan Group	25.7	39.7	9.9	11.2
Prednisone Group	36.6	56.6	23.2	23.4

"BVAS=Birmingham Vasculitis Activity Score~UACR=Urinary albumin:creatinine ratio" (NCT02994927)
Timeframe: Baseline, Week 4, 26 and 52

In Subjects With Renal Disease at Baseline (Based in the BVAS Renal Component), the Change in eGFR From Baseline Over 52 Weeks

Intervention	Percentage change (Least Squares Mean)
	Week 4	Week 26	Week 52
Avacopan Group	-40	-63	-74
Prednisone Group	0	-70	-77

"Change from baseline in kidney function, as measured by eGFR (based on the MDRD equation), was measured in subjects with renal disease based on the BVAS renal component.~eGFR=estimated glomerular filtration rate~BVAS=Birmingham Vasculitis Activity Score~MDRD=Modification of Diet in Renal Disease" (NCT02994927)
Timeframe: Baseline, Week 26 and 52

In Subjects With Renal Disease at Baseline (Based in the BVAS Renal Component), the Percent Change in Urinary MCP-1:Creatinine Ratio From Baseline Over 52 Weeks

Intervention	Change in eGFR (mL/min/1.73 m^2) (Least Squares Mean)
	Week 26	Week 52
Avacopan Group	5.8	7.3
Prednisone Group	2.9	4.1

"BVAS=Birmingham Vasculitis Activity Score~MCP-1=monocyte chemoattractant protein-1" (NCT02994927)
Timeframe: Baseline, Week 26 and 52

Number of Subjects Where a Relationship Between Avacopan/Placebo, Glucocorticoid Use, Cyclophosphamide, Rituximab, and Azathioprine or Mycophenolate Use to an AE Was Determined by the Investigator

Intervention	Percentage change (Least Squares Mean)
	Week 26	Week 52
Avacopan Group	-67	-73
Prednisone Group	-64	-71

AE=Adverse Event (NCT02994927)
Timeframe: From day 1 throughout the study period (day 421/week 60)

Subject Incidence of Treatment-emergent SAEs, AEs, and Withdrawals Due to AEs

Intervention	Participants (Count of Participants)
	Relationship of avacopan/placebo to an AE	Relationship of glucocorticoid use to an AE	Relationship of cyclophosphamide IV use to an AE	Relationship of oral cyclophosphamide use to an AE	Relationship of rituximab use to an AE	Relationship of azathioprine use to an AE	Relationship of mycophenolate use to an AE
Avacopan Group	100	107	31	8	50	28	6
Prednisone Group	103	131	30	4	61	35	9

"AEs=Adverse events~SAEs=Serious adverse events~TEAE=Treatment-emergent adverse event" (NCT02994927)
Timeframe: From day 1 throughout the study period (day 421/week 60)

Disease Improvement

Intervention	Number (Number)
	Number of subjects with at least one TEAE	Number of TEAEs	Number of subjects with SAEs	Number of SAEs	Subjects with TEAE leading to discontinuation
Avacopan Group	164	1779	70	116	27
Prednisone Group	161	2139	74	166	28

"Disease improvement was measured by a reduction in the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG).~The BVAS/WG is a validated disease activity index. The BVAS/WG is designed to document new or worsening clinically active vasculitis and consists of a set of items divided into nine organ based systems. BVAS/WG scores range from 0 to 63." (NCT00468208)
Timeframe: Measured monthly until common closing or early termination, up to 3 years and 4 months.

Disease Relapse

Intervention	participants (Number)
Open-label Abatacept	18

"Disease relapse was measured by a rise in the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) of greater than or equal to 1 after achieving remission.~The BVAS/WG is a validated disease activity index. The BVAS/WG is designed to document new or worsening clinically active vasculitis and consists of a set of items divided into nine organ based systems. BVAS/WG scores range from 0 to 63." (NCT00468208)
Timeframe: Measured monthly until common closing or early termination, up to 3 years and 4 months.

Disease Remission

Intervention	participants (Number)
Open-label Abatacept	3

"Disease remission was measured by a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) of 0.~The BVAS/WG is a validated disease activity index. The BVAS/WG is designed to document new or worsening clinically active vasculitis and consists of a set of items divided into nine organ based systems. BVAS/WG scores range from 0 to 63." (NCT00468208)
Timeframe: Measured monthly until common closing or early termination,up to 3 years and 4 months.

Meeting Common Closing

Intervention	participants (Number)
Open-label Abatacept	16

The number of subjects that reached the common closing date. (NCT00468208)
Timeframe: Number assessed at the time of common closing, up to 3 years and 4 months.

Safety of Abatacept - Number of Participants With Adverse Events

Intervention	participants (Number)
Open-label Abatacept	14

"This study examined the safety profile of this agent when used in Wegener's granulomatosis. Information was gathered on all adverse events with specific events being identified in the protocol for analysis that included the following:~Infection~Infusion reactions~Cytopenias~Transaminase elevation~Skin reactions~GI side effects~Malignancy~All adverse events were reportable for this study." (NCT00468208)
Timeframe: Measured continuously from the screening visit through to the 6 month post-treatment study visit, up to 3 years and 4 months.

Disease Remission

Intervention	participants (Number)
	Serious adverse events	Non-serious adverse events	Infection	Infusion related (systemic)	Infusion related( intravenous site reaction)	Cytopenia
Open-label Abatacept	7	16	14	1	1	4

A Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) score of 0 with prednisone taper successfully completed at six months. The BVAS/WG is a validated disease activity index. The BVAS/WG is designed to document new or worsening clinically active vasculitis and consists of a set of items divided into nine organ based systems. BVAS/WG scores range from 0 to 63, with higher scores indicating more active disease. (NCT00104299)
Timeframe: 6 months post-randomization

Percentage of Participants Who Have a BVAS/WG Score of 0 and Have Successfully Completed the Glucocorticoid Taper by 6 Months Post-randomization

Intervention	Participants (Number)
Rituximab	63
Control Group	52

"The 2-sided 95% CI of the percentage of participants who have a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)[1] of 0 and have successfully completed the glucocorticoid taper by 6 months post-randomization and the 2-sided 95% CI of the difference between two arms for assessing the superiority of rituximab to control~[1] The BVAS/WG is a disease activity index designed to document new or worsening clinically active vasculitis consisting of items divided into 9 organ based systems. BVAS/WG scores range from 0 to 63, with higher scores indicating more active disease" (NCT00104299)
Timeframe: 6 months post-randomization

Number of Subjects Experiencing Serious Adverse Events

Intervention	participants (Number)
Rituximab	62
Control Group	51

Number of subjects according to originally received treatment that experienced a serious adverse event through 18 months post-randomization or prior to being censored from analyses due to crossover, switching to open-label treatment, or best medical judgment for censor. Events are categorized by coded system organ classes (SOC). Within each SOC, a participant was counted once if the participant reported one or more events coded to that SOC. (NCT00104299)
Timeframe: Randomization to censor at Crossover, Open-label or Best Medical Judgment (up to 18 months post-randomization)

Rate of Selected Adverse Events Experienced by Participants Receiving Rituximab Versus Those Receiving Conventional Therapy

Intervention	participants (Number)
	# Participants with at least one SAE	Blood and Lymphatic System Disorders	Cardiac Disorders	Eye Disorders	Gastrointestinal Disorders	General Disorders and Administration Site	Immune System Disorders	Infections and Infestations	Injury, Poisoning, and Procedural Complications	Investigations	Metabolism and Nutrition Disorders	Musculoskeletal and Connective Tissue Disorders	Neoplasms Benign, Malignant, and Unspecified	Nervous System Disorders	Pregnancy, Puerperium, and Perinatal Conditions	Psychiatric Disorders	Renal and Urinary Disorders	Respiratory, Thoracic, and Mediastinal Disorders	Vascular Disorders
Control Group	37	5	2	1	1	3	2	12	0	0	2	3	2	0	0	1	3	8	7
Rituximab	42	4	2	1	4	5	2	12	2	2	2	2	1	1	1	1	4	8	1

The adverse event rate for the following events considered related to vasculitis: Death; Grade 2 or higher leukopenia or thrombocytopenia; Grade 3 or higher infections; Hemorrhagic cystitis (grade 2 or lower needs confirmation by cytoscopy); Malignancy; Venous thromboembolic event (deep venous thrombosis or pulmonary embolism); Hospitalization resulting either from the disease or from a complication due to study treatment; Infusion reactions (within 24 hours of infusion) that result in the cessation of further infusions (including cytokine release allergic reaction); Cerebrovascular accident (NCT00104299)
Timeframe: Through common close-out (defined as 18 months after the last participant is enrolled in the trial)

The Duration of Complete Remission (BVAS=0, Off Glucocorticoids), the Time to Limited and/or Severe Flare After Remission in the Two Treatment Groups

Intervention	participants (Number)
	Death	Grade 2 or Higher Leukopenia	Grade 2 or Higher Thrombocytopenia	Grade 3 or Higher Infections	Hemorrhagic Cystitis (Grade 2 or Lower)	Malignancy	Venous Thromboembolic Event	Hospitalization Resulting from the Disease	Cerebrovascular Accident (CVA)	Infusion Reactions Leading to Infusion Disc.
Control Group	2	23	1	16	1	2	8	7	1	0
Rituximab	2	7	4	18	2	5	6	16	1	1

"Duration of complete remission is defined as a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)[1] of 0 and a completing taper of Prednisone to the first flare, BVAS/WG score of greater than 0, or an increase in Prednisone dosing.~[1] The BVAS/WG is a disease activity index designed to document new or worsening clinically active vasculitis consisting of items divided into 9 organ based systems. BVAS/WG scores range from 0 to 63, with higher scores indicating more active disease" (NCT00104299)
Timeframe: 18 months post-randomization

The Duration of Remission (BVAS=0), the Time to Limited and/or Severe Flare After Remission in the Two Treatment Groups

Intervention	Days (Number)
	25% Quartile (95%CI)	50% Quartile (95%CI)	75% Quartile (95%CI)
Control Group	230	NA	NA
Rituximab	243	NA	NA

Duration of remission is defined as a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)[1] of 0 and a completing taper of glucocorticoid by 6 months post-randomization to the first flare, BVAS/WG score of greater than 0, or an increase in Prednisone dosing. (NCT00104299)
Timeframe: 18 months post-randomization

Time to Complete Remission (BVAS=0, Off Glucocorticoids) From the Visit 1 Baseline Visit in the Two Treatment Groups

Intervention	Days (Number)
	25% Quartile (95%CI)	50% Quartile (95%CI)	75% Quartile (95%CI)
Control Group	168	NA	NA
Rituximab	246	NA	NA

"Time to complete remission is defined as the number of days from baseline visit (Visit 1) to a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)[1] of 0 and completing taper of glucocorticoid by 6 months post-randomization.~[1] The BVAS/WG is a disease activity index designed to document new or worsening clinically active vasculitis consisting of items divided into 9 organ based systems. BVAS/WG scores range from 0 to 63, with higher scores indicating more active disease" (NCT00104299)
Timeframe: 18 months post-randomization

Time to Remission (BVAS=0) From the Visit 1 Baseline Visit in the Two Treatment Groups

Intervention	Days (Number)
	25% Quartile (95%CI)	50% Quartile (95%CI)	75% Quartile (95%CI)
Control Group	177	183	266
Rituximab	176	180	189

"Time to complete remission is defined as the number of days from baseline visit (Visit 1) to a Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG)[1] of 0.~[1] The BVAS/WG is a disease activity index designed to document new or worsening clinically active vasculitis consisting of items divided into 9 organ based systems. BVAS/WG scores range from 0 to 63, with higher scores indicating more active disease" (NCT00104299)
Timeframe: 18 months post-randomization

Composite of Disease Relapse (Defined a BVAS/WG ≥ 2) and Serious Adverse Events

Intervention	Days (Number)
	25% Quartile (95%CI)	50% Quartile (95%CI)	75% Quartile (95%CI)
Control Group	29	43	112
Rituximab	30	57	119

Number of disease relapse added with the number of SAE in each group (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Mean Number of Rituximab Infusions Per Subject

Intervention	Number of events (Number)
B Cell Reconstitution	27
Serologic ANCA Flare	36

The rituximab utilization was measured in how many times a subject received Rituximab throughout the study which was then averaged for all subjects in each treatment arm, including those who did not receive any infusion. (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Number of Major Relapses Defined as a BVAS/WG ≥ 3

Intervention	Infusions per subject (Mean)
B Cell Reconstitution	3.6
Serologic ANCA Flare	0.5

The Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG ) is a form with 34 predefined items grouped into 9 organ systems. The items are clinical features observed in patients with active ANCA vasculitis. Each item has a specified weight of either 3 or 1, depending on whether it reflects major or minor disease activity. The total BVAS/WG score is the weighted sum of individual manifestations that are present and believed to be due to active ANCA vasculitis. Higher scores reflect more active disease. BVAS/WG scores range from 0 to 64. (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Number of Patients Affected by Serious Adverse Events

Intervention	number of events (Number)
B Cell Reconstitution	4
Serologic ANCA Flare	7

Number of patients with serious adverse events (SAEs), including all episodes of late onset neutropenia (LON). SAE are defined in the Serious adverse event section. Serious adverse events were reported over the entire study period (5.5 years) (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Number of Patients With Hypogammaglobulinemia

Intervention	Participants (Count of Participants)
B Cell Reconstitution	15
Serologic ANCA Flare	14

Hypogammaglobulinemia defined as an IgG < 250mg/dL (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Patient Survival

Health-related Quality of Life as Assessed by the Short Form Health Survey (SF-36) Scores

Intervention	Participants (Count of Participants)
B Cell Reconstitution	1
Serologic ANCA Flare	0

The Short Form (36) Health Survey is a 36-item, patient-reported survey of patient health. The SF-36 is a measure of health status and is commonly used in health economics as a variable in the quality-adjusted life year calculation to determine the cost-effectiveness of a health treatment. The SF-36 consists of eight scaled scores, which are the weighted sums of the questions in their section. Each scale is directly transformed into a 0-100 scale. The lower the score the more disability. The higher the score the less disability i.e., a score of zero is equivalent to maximum disability and a score of 100 is equivalent to no disability. (NCT02749292)
Timeframe: Assessed throughout the study period, every 6 months unless such time point was not reached or was missed by the patient. Median follow-up period is of 4.1 years (IQR, 2.5 - 5.0)

Health-related Quality of Life as Assessed by the Short Form Health Survey (SF-36) Scores

Number of Infections

Number of infections mild and severe, whether they were treated or not with antibiotics (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Number of Patients With Disease Relapse(s) as Defined by a Birmingham Vasculitis Activity Score for Wegner's Granulomatosis (BVAS/WG) ≥ 2

Relapses recording period was from 6/1/2016 to 12/31/2021. The outcome was reported as the number of participants with disease relapse who had either positive ANCA titers specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). The Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG ) is a form with 34 predefined items grouped into 9 organ systems. The items are clinical features observed in patients with active ANCA vasculitis. Each item has a specified weight of either 3 or 1, depending on whether it reflects major or minor disease activity. The total BVAS/WG score is the weighted sum of individual manifestations that are present and believed to be due to active ANCA vasculitis. Higher scores reflect more active disease. BVAS/WG scores range from 0 to 64. (NCT02749292)
Timeframe: Median follow-up period of 4.1 years (IQR, 2.5 - 5.0)

Organ Damage as Assessed by the Vasculitis Damage Index (VDI).

The Vasculitis Damage Index (VDI) is a validated formal assessment tool in ANCA-associated vasculitis clinical trials. The VDI distinguishes vasculitis-induced chronic damage from active inflammation or persistent disease. Each item represents a disease manifestation and is given a score (of 1) if present for at least 3 months. Neither the cause of damage (vasculitis vs treatment) nor an ongoing activity are taken into consideration. The VDI includes 64 items categorized into 11 groups (by organ system) and the scored items are summed to give a total score ranging from 0 to 64. A higher score means more accrued damage. (NCT02749292)
Timeframe: 3 years starting at inclusion

Health-related Quality of Life Using the SF-36 Mental Composite

The 36-Item Short Form Health Survey (SF-36) is a set of generic, coherent, and easily administered quality-of-life measures. Scores for the scale range from 0-100 and transformed to have a mean of 50 and SD of 10 in the reference population, with higher scores indicating a better Health-related Quality of Life. (NCT01697267)
Timeframe: 12 months

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Infection Rates

Infection (treated with intravenous or oral antibiotics) rates (NCT01697267)
Timeframe: Up to 4 years

Severe Adverse Event Rate

Combined Damage Assessment Score (Disease Related Damage Assessment)

Cumulative accrual of damage as measured by the combined damage assessment score (CDA). Each persistent or new occurrence of damage is given a score of 1. The cumulative accrual of damage is obtained by summing across the different types of damage to get an overall score (max score = 64). (NCT01697267)
Timeframe: data in Rows represent the change from randomization (month 4) to months 12, 24, 36, and 48.

Cumulative GC Exposure

Cumulative glucocorticoid (GC) exposure during the trial. The trial had a common close out date when the final patient reached month 36 in the trial. Patients were followed until month 48 or the common close out date, whichever happened sooner. Therefore, follow up varied between 36 and 48 months. Cumulative glucocorticoid exposure is presented as a dose in mg for during the treatment period (up to month 24) and across the whole trial (until month 48 or common close out when the final patient reached month 36). (NCT01697267)
Timeframe: Up to 48 months

Number of Participants in Remission at 24 and 48 Months

Proportion of patients who maintain remission at 24 and 48 months (NCT01697267)
Timeframe: 24 and 48 months

Relapse-free Survival

The primary efficacy outcome measure of the trial is relapse-free survival, where a relapse is either major or minor. The primary analysis will be a Cox regression model adjusted for the stratification factors (ANCA type, relapse severity and prednisone induction regimen) for the difference in the distribution of relapse-free survival between the rituximab arm and the azathioprine (control) arm (two-sided at α-level of 5%). (NCT01697267)
Timeframe: Any patients who have not relapsed at up to a maximum of 4 years will be censored.

Intervention	score on a scale (Mean)
	Physical functioning at 6 months	Role functioning/physical at 6 months	Role functioning/emotional at 6 months	Energy/fatigue at 6 months	Emotional well-being at 6 months	Social functioning at 6 months	Pain at 6 months	General health at 6 months	Physical functioning at 36 months	Role functioning/physical at 36 months	Role functioning/emotional at 36 months	Energy/fatigue at 36 months	Emotional well-being at 36 months	Social functioning at 36 months	Pain at 36 months	General health at 36 months	Physical functioning at 12 months	Role functioning/physical at 12 months	Role functioning/emotional at 12 months	Energy/ fatigue at 12 months	Emotional well-being at 12 months	Social functioning at 12 months	Pain at 12 months	General health at 12 months	Physical functioning at 18 months	Role functioning/physical at 18 months	Role functioning/emotional at 18 months	Energy/fatigue at 18 months	Emotional well-being at 18 months	Social functioning at 18 months	Pain at 18 months	General health at 18 months	Physical functioning at 24 months	Role functioning/physical at 24 months	Role functioning/emotional at 24 months	Energy/fatigue at 24 months	Emotional well-being at 24 months	Social functioning at 24 months	Pain at 24 months	General health at 24 months	Physical functioning at 30 months	Role functioning/physical at 30 months	Role functioning/emotional at 30 months	Energy/fatigue at 30 months	Emotional well-being at 30 months	Social functioning at 30 months	Pain at 30 months	General health at 30 months	Physical functioning at 42 months	Role functioning/physical at 42 months	Role functioning/emotional at 42 months	Energy/fatigue at 42 months	Emotional well-being at 42 months	Social functioning at 42 months	Pain at 42 months	General health at 42 months	Physical functioning at 48 months	Role functioning/physical at 48 months	Role functioning/emotional at 48 months	Energy/fatigue at 48 months	Emotional well-being at 48 months	Social functioning at 48 months	Pain at 48 months	General health at 48 months	Physical functioning at 60 months	Role functioning/physical at 60 months	Role functioning/emotional at 60 months	Energy/fatigue at 60 months	Emotional well-being at 60 months	Social functioning at 60 months	Pain at 60 months	General health at 60 months
Serologic ANCA Flare	82	65	75	67	81	88	82	63	88	79	82	73	84	95	87	70	82	63	68	65	81	86	80	63	82	62	74	68	81	92	82	67	88	71	80	76	85	94	82	70	88	72	80	72	84	94	83	70	82	60	76	70	85	87	82	63	95	100	100	75	85	100	80	75	92	100	89	71	83	92	83	70

Intervention	number of events (Number)
	Serious Adverse Events- Infections	Adverse Events- Infections
B Cell Reconstitution	12	72
Serologic ANCA Flare	6	59

Intervention	score on a scale (Mean)
	VDI at inclusion	VDI at 3 years
B Cell Reconstitution	1.27	1.42
Serologic ANCA Flare	1.07	1.08

Intervention	score on a scale (Mean)
	Randomisation to month 12	Randomisation to month 24	Randomisation to month 36	Randomisation to month 48
Azathioprine Maintenance	0.337	0.533	0.899	1.38
Rituximab Maintenance	0.275	0.571	0.676	1.09

Intervention	mg (Mean)
	Overall (randomisation to end of trial)	Maintenance treatment period (randomisation to month 24)
Azathioprine Maintenance	4780	2426
Rituximab Maintenance	3717	2184

Intervention	Participants (Count of Participants)
	Month 24	Month 48
Azathioprine Maintenance	70	44
Rituximab Maintenance	73	54

Intervention	participants (Number)
	Total number of patients with a relapse	Total number of patients with a relapse during treatment	Total number of patients with a relapse post treatment
Azathioprine Maintenance	60	32	28
Rituximab Maintenance	38	13	25

Article	Year
Granulomatosis with polyangiitis with isolated orbital involvement in children: a case report successfully treated with Rituximab and review of literature. Acta reumatologica portuguesa, 2019, 07-08, Volume: 44, Issue:Jul-Sep (3 Topics: Adolescent; Anti-Inflammatory Agents; Female; Granulomatosis with Polyangiitis; Humans; Immunosuppre	2019
Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist. Pediatric rheumatology online journal, 2019, Jun-26, Volume: 17, Issue:1 Topics: Adult; Anti-Inflammatory Agents; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Azathio	2019
Strategies to prevent the neuropsychiatric side-effects of corticosteroids: a case report and review of the literature. Current opinion in organ transplantation, 2014, Volume: 19, Issue:2 Topics: Adult; Amines; Benzodiazepines; Cyclohexanecarboxylic Acids; Drug Therapy, Combination; Female; Gaba	2014
Benign pneumatosis intestinalis in a pediatric patient with multiple risk factors including granulomatosis with polyangiitis: a case report and review of the literature. Seminars in arthritis and rheumatism, 2015, Volume: 44, Issue:4 Topics: Adolescent; Anti-Bacterial Agents; Antifungal Agents; Comorbidity; Drug Therapy, Combination; Female	2015
Cyclophosphamide as induction therapy for Wegener's granulomatosis and microscopic polyangiitis. Clinical and experimental immunology, 2011, Volume: 164 Suppl 1 Topics: Antibodies, Monoclonal, Murine-Derived; Cyclophosphamide; Female; Granulomatosis with Polyangiitis;	2011
Urogenital manifestations in Wegener granulomatosis: a study of 11 cases and review of the literature. Medicine, 2012, Volume: 91, Issue:2 Topics: Adult; Aged; Constriction, Pathologic; Cystostomy; Follow-Up Studies; Genital Diseases, Male; Glucoc	2012
Cutaneous Wegener's granulomatosis: a variant or atypical localized form? The Australasian journal of dermatology, 2003, Volume: 44, Issue:2 Topics: Aged; Antibodies, Antineutrophil Cytoplasmic; Biopsy, Needle; Cyclophosphamide; Drug Therapy, Combin	2003
Protracted superficial Wegener's granulomatosis. The Australasian journal of dermatology, 2003, Volume: 44, Issue:3 Topics: Adult; Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Azathioprine; Biopsy; Drug	2003
[Wegener's granulomatosis (morbus Wegener)]. Praxis, 2003, Nov-12, Volume: 92, Issue:46 Topics: Adult; Anti-Infective Agents; Anti-Inflammatory Agents; Azathioprine; Cyclophosphamide; Diagnosis, D	2003
Vasculitis of the nervous system. Current opinion in neurology, 2004, Volume: 17, Issue:3 Topics: Azathioprine; Connective Tissue Diseases; Cyclophosphamide; Diagnosis, Differential; Giant Cell Arte	2004
Bilateral simultaneous central retinal artery occlusions in wegener granulomatosis. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society, 2005, Volume: 25, Issue:1 Topics: Antibodies, Antineutrophil Cytoplasmic; Dose-Response Relationship, Drug; Female; Glucocorticoids; G	2005
Wegener's granulomatosis: the current understanding. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics, 2005, Volume: 100, Issue:3 Topics: Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Cyclophosphamide; Dental Care for	2005
[(Micro-)hematuria (in adults). Main symptoms: evidence of erythrocytes in urine sediment]. Praxis, 2005, Sep-21, Volume: 94, Issue:38 Topics: Adult; Age Factors; Anti-Infective Agents; Anti-Inflammatory Agents; Antibodies, Antineutrophil Cyto	2005
Wegener granulomatosis: a case report and update. Southern medical journal, 2006, Volume: 99, Issue:9 Topics: Antibodies, Antineutrophil Cytoplasmic; Cyclophosphamide; Drug Administration Schedule; Female; Gluc	2006
[Treatment of ANCA-associated vascularitides]. Presse medicale (Paris, France : 1983), 2007, Volume: 36, Issue:5 Pt 2 Topics: Adrenal Cortex Hormones; Antibodies, Antineutrophil Cytoplasmic; Antibodies, Monoclonal; Antibodies,	2007
[Wegener's granulomatosis in ENT: clinical presentation, diagnosis and therapy]. Laryngo- rhino- otologie, 2007, Volume: 86, Issue:11 Topics: Adolescent; Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Anticoagulants; Cyclop	2007
Vasculitis: a collection of pearls and myths. Rheumatic diseases clinics of North America, 2007, Volume: 33, Issue:4 Topics: Antirheumatic Agents; Behcet Syndrome; Churg-Strauss Syndrome; Cyclophosphamide; Giant Cell Arteriti	2007
Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Annals of internal medicine, 1983, Volume: 98, Issue:1 Topics: Adolescent; Adult; Aged; Azathioprine; Clinical Trials as Topic; Cyclophosphamide; Drug Administrati	1983
Wegener's granulomatosis. Otolaryngologic clinics of North America, 1982, Volume: 15, Issue:3 Topics: Cyclophosphamide; Ear Diseases; Eye Diseases; Granulomatosis with Polyangiitis; Humans; Laryngeal Di	1982
Salivary gland involvement in Wegener's granulomatosis. A case report and review of the literature. Oral surgery, oral medicine, and oral pathology, 1994, Volume: 77, Issue:3 Topics: Adult; Antibodies, Antineutrophil Cytoplasmic; Autoantibodies; Biomarkers; Cyclophosphamide; Female;	1994
Exacerbation of Wegener's granulomatosis during pregnancy: report of a case with tracheal stenosis and literature review. The Journal of rheumatology, 1994, Volume: 21, Issue:6 Topics: Adult; Constriction, Pathologic; Female; Granulomatosis with Polyangiitis; Humans; Laser Therapy; Pr	1994
Wegener's granulomatosis: case report and review of the literature. Rhinology, 1994, Volume: 32, Issue:2 Topics: Adult; Antibodies, Antineutrophil Cytoplasmic; Autoantibodies; Biomarkers; Cyclophosphamide; Diagnos	1994
Wegener's granulomatosis. The great masquerade: a clinical presentation and literature review. The Journal of otolaryngology, 1993, Volume: 22, Issue:2 Topics: Adult; Biopsy; Cyclophosphamide; Diagnosis, Differential; Granulomatosis with Polyangiitis; Humans;	1993
[Immunosuppressive therapy of vasculitis: current aspects and perspectives]. Recenti progressi in medicina, 1996, Volume: 87, Issue:3 Topics: Adrenal Cortex Hormones; Anti-Inflammatory Agents; Behcet Syndrome; Cyclophosphamide; Drug Therapy,	1996
Wegener's granulomatosis complicating pregnancy: presentation of two patients and review of the literature. Clinical nephrology, 1996, Volume: 46, Issue:5 Topics: Adult; Cyclophosphamide; Drug Therapy, Combination; Female; Granulomatosis with Polyangiitis; Humans	1996
Successful treatment of Wegener's granulomatosis during pregnancy: a case report and review of the medical literature. Chest, 1998, Volume: 113, Issue:3 Topics: Adult; Cyclophosphamide; Female; Glucocorticoids; Granulomatosis with Polyangiitis; Humans; Immunosu	1998
[Treatment of ANCA-positive systemic vasculitis with intravenous immunoglobins]. La Revue de medecine interne, 1999, Volume: 20 Suppl 4 Topics: Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Arteritis; Churg-Strauss Syndrome;	1999
Otitis media as a sign of Wegener's granulomatosis in childhood. Acta oto-laryngologica. Supplementum, 2000, Volume: 543 Topics: Administration, Oral; Adolescent; Adult; Anti-Infective Agents, Urinary; Anti-Inflammatory Agents; A	2000
Radiation therapy in lymphomatoid granulomatosis. Cancer, 1978, Volume: 42, Issue:6 Topics: Cyclophosphamide; Female; Granuloma; Granulomatosis with Polyangiitis; Humans; Lymphoma; Lymphoproli	1978
Midline granuloma and Wegener's granulomatosis: clinical & therapeutic considerations. The Journal of rheumatology, 1976, Volume: 3, Issue:3 Topics: Adrenal Cortex Hormones; Adult; Antineoplastic Agents; Azathioprine; Cyclophosphamide; Drug Therapy,	1976
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prednisone and Granulomatosis, Wegener's

Research Excerpts

Research

Studies (349)

Authors

Clinical Trials (25)

Trial Overview

Trial Outcomes

Number of Subjects Experiencing a Relapse After Previously Achieving BVAS=0 During the Study

Number of Subjects With Clinically Significant ECG Changes From Baseline

Percentage of Participants With BVAS of 0 at Week 4, Regardless of Whether the Subjects Received Glucocorticoids During This Period of Time and Based on Assessment by the Blinded AC

Percentage of Subjects Achieving Disease Remission at Week 26

Percentage of Subjects Achieving Sustained Disease Remission at Week 52

Percentage of Subjects and Time to Experiencing a Relapse After Previously Achieving BVAS=0 at Any Time During the Treatment Period

Percentage of Subjects and Time to Experiencing a Relapse After Previously Achieving Remission at Week 26 in the Study

Certain Safety Endpoints of Interest: Infections, Hepatic System Abnormalities, WBC Count Decreases, and Hypersensitivity

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (1/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (2/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (3/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (4/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Hematology (5/5)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Serum Chemistry (1/2)

Change From Baseline and Shifts From Baseline in All Safety Laboratory Parameters - Serum Chemistry (2/2)

Change From Baseline in Vital Signs (1/5)

Change From Baseline in Vital Signs (2/5)

Change From Baseline in Vital Signs (3/5)

Change From Baseline in Vital Signs (4/5)

Change From Baseline in Vital Signs (5/5)

Change From Baseline Over 52 Weeks in Health-related Quality of Life as Measured by the Domains and Component Scores of the SF-36v2 and EQ-5D-5L VAS and Index

Change in the VDI From Baseline Over 52 Weeks, Including the Week 26 and Week 52 Time Points

Glucocorticoid-induced Toxicity as Measured by Change From Baseline Over the First 26 Weeks in the GTI

In Subjects With Renal Disease and Albuminuria at Baseline (Based in the BVAS Renal Component), the Percent Change in UACR From Baseline Over 52 Weeks

In Subjects With Renal Disease at Baseline (Based in the BVAS Renal Component), the Change in eGFR From Baseline Over 52 Weeks

In Subjects With Renal Disease at Baseline (Based in the BVAS Renal Component), the Percent Change in Urinary MCP-1:Creatinine Ratio From Baseline Over 52 Weeks

Number of Subjects Where a Relationship Between Avacopan/Placebo, Glucocorticoid Use, Cyclophosphamide, Rituximab, and Azathioprine or Mycophenolate Use to an AE Was Determined by the Investigator

Subject Incidence of Treatment-emergent SAEs, AEs, and Withdrawals Due to AEs

Disease Improvement

Disease Relapse

Disease Remission

Meeting Common Closing

Safety of Abatacept - Number of Participants With Adverse Events

Disease Remission

Percentage of Participants Who Have a BVAS/WG Score of 0 and Have Successfully Completed the Glucocorticoid Taper by 6 Months Post-randomization

Number of Subjects Experiencing Serious Adverse Events

Rate of Selected Adverse Events Experienced by Participants Receiving Rituximab Versus Those Receiving Conventional Therapy

The Duration of Complete Remission (BVAS=0, Off Glucocorticoids), the Time to Limited and/or Severe Flare After Remission in the Two Treatment Groups

The Duration of Remission (BVAS=0), the Time to Limited and/or Severe Flare After Remission in the Two Treatment Groups

Time to Complete Remission (BVAS=0, Off Glucocorticoids) From the Visit 1 Baseline Visit in the Two Treatment Groups

Time to Remission (BVAS=0) From the Visit 1 Baseline Visit in the Two Treatment Groups

Composite of Disease Relapse (Defined a BVAS/WG ≥ 2) and Serious Adverse Events

Mean Number of Rituximab Infusions Per Subject

Number of Major Relapses Defined as a BVAS/WG ≥ 3

Number of Patients Affected by Serious Adverse Events

Number of Patients With Hypogammaglobulinemia

Patient Survival

Health-related Quality of Life as Assessed by the Short Form Health Survey (SF-36) Scores

Health-related Quality of Life as Assessed by the Short Form Health Survey (SF-36) Scores

Number of Infections

Number of Patients With Disease Relapse(s) as Defined by a Birmingham Vasculitis Activity Score for Wegner's Granulomatosis (BVAS/WG) ≥ 2

Organ Damage as Assessed by the Vasculitis Damage Index (VDI).

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Mental Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Health-related Quality of Life Using the SF-36 Physical Composite

Infection Rates

Severe Adverse Event Rate

Combined Damage Assessment Score (Disease Related Damage Assessment)

Cumulative GC Exposure

Number of Participants in Remission at 24 and 48 Months

Relapse-free Survival

Reviews

Trials

Other Studies