mycophenolic-acid and Hematuria

IgA nephropathy (IgAN) is a leading cause of CKD and renal failure. Recent international collaborative efforts have led to important discoveries that have improved our understanding of some of the key steps involved in the immunopathogenesis of IgAN. Furthermore, establishment of multicenter networks has contributed to rigorous design and execution of clinical trials that have provided important insights regarding immunotherapy in IgAN. In this article, we review emerging developments in clinical and translational IgAN research and describe how these novel findings will influence future strategies to improve the outcome of patients with IgAN.

Topics: Adrenal Cortex Hormones; Conservative Treatment; Drug Therapy, Combination; Glomerulonephritis, IGA; Hematuria; Humans; Immunologic Factors; Mycophenolic Acid; Phenotype; Proteinuria; Renal Insufficiency, Chronic; Rituximab

The presence of renal noninflammatory necrotizing vasculopathy (NNV) is often associated with a severe form of lupus nephritis (LN), which is unresponsive to standard therapy. We conducted a 6-month randomized, prospective, open-label trial comparing mycophenolate mofetil (MMF) (1.5-2.0 g/day) with monthly i.v. cyclophosphamide (CTX) (0.75-1.0 g/m2) as induction therapy for class IV LN with NNV. The primary and second end points were complete remission (CR) and partial remission (PR), respectively. Of 20 patients recruited, nine were randomly assigned to MMF and 11 to CTX. The baseline characteristics between groups were not significant. CR was achieved in four patients (44.4%) receiving MMF and in none of the patients receiving CTX (P = 0.026). PR was achieved in two patients (22.2%) in the MMF group and three patients (27.2%) in the CTX group. The total remission rate (CR + PR) in the MMF and CTX group was 66.6 and 27.2%, respectively (P = 0.17). MMF was more effective than i.v. CTX in reducing proteinuria and haematuria. Adverse events were significantly less frequent with MMF than with CTX (P = 0.028). MMF was superior to i.v. CTX in inducing CR of LN with NNV and had a more favourable safety profile.

Topics: Adolescent; Adult; Cyclophosphamide; Dose-Response Relationship, Drug; Drug Therapy, Combination; Follow-Up Studies; Glucocorticoids; Hematuria; Humans; Immunosuppressive Agents; Infusions, Intravenous; Lupus Nephritis; Middle Aged; Mycophenolic Acid; Prospective Studies; Proteinuria; Remission Induction; Vascular Diseases

While the use of different immunosuppressants has been investigated in immunoglobulin A nephropathy, further investigation is needed to assess the effect of a regimen of mycophenolate mofetil combined with a short course of glucocorticosteroids in the subset of patients with histologically active features. We compared the efficacy and safety of a combined regimen of mycophenolate mofetil and glucocorticosteroids to a conventional regimen of glucocorticosteroids alone in patients with immunoglobulin A nephropathy who have active lesions and major urinary abnormalities.. This retrospective study involved 30 immunoglobulin A nephropathy patients with active histological lesions, 15 of whom were treated with both mycophenolate mofetil 2 g/day for 6 months and 3 pulses of 15 mg/kg methylprednisolone, followed by a short tapering schedule of oral prednisone. The control group was made up of the remaining 15 clinically- and histologically-matched patients treated with glucocorticosteroids alone according to a validated schedule, i.e., 1 g of methylprednisolone given intravenously for 3 consecutive days, followed by oral prednisone 0.5 mg/kg every other day for 6 months. At diagnosis, all patients had urinary protein excretion > 1 g/24 h and microscopic hematuria.. At the end of the first year of follow-up (30 patients) and after 5 years (17 patients), there were no differences between the two groups in terms of urinary abnormalities and functional parameters. Both regimens achieved a statistically significant decrease in 24-h urinary protein excretion (p < 0.001) and a reduction of microscopic hematuria. However, the mycophenolate mofetil-based regimen allowed a cumulative sparing dose of 6 g of glucocorticosteroids.. In this single center study on immunoglobulin A nephropathy patients with active lesions and major urinary abnormalities and at increased risk of glucocorticosteroid-related complications, a mycophenolate mofetil-based regimen demonstrated similar outcomes in terms of complete response and relapse (at 1 and 5 years) compared to a conventional glucocorticosteroid-based protocol, while achieving a consistent reduction of glucocorticosteroid cumulative dose.

Topics: Drug Therapy, Combination; Glomerulonephritis, IGA; Hematuria; Humans; Immunosuppressive Agents; Methylprednisolone; Mycophenolic Acid; Prednisone; Retrospective Studies

There is no standard recommendation for IgA nephropathy treatment in children.. This is a retrospective study. From 2012 to 2020, newly diagnosed primary IgAN followed up for at least 1 year were enrolled. The correlation of MESTC scores and clinical index including proteinuria, gross hematuria and renal dysfunction was analyzed. Treatment and clinical response of 6 month, 1year and 3 year at follow up were also analyzed. Complete renal remission was calculated with Kaplan-Meier analysis.. The median follow up was 36 months, from 12 months to 87months in 40 IgAN children. Angiotensin-converting enzyme inhibitor (ACEI) was applied to all patients. 30% received ACEI alone; 15% received glucocorticoids; 37.5% received glucocorticoids plus cyclophosphamide, 17.5% received glucocorticoids plus mycophenolate mofetil. Individuals with diffuse mesangial hypercellularity (M1) were more likely to have nephrotic range proteinuria compared to patients with M0 (80% vs. 20%, P < 0.01). Complete renal remission at 6-month, 1-year and 3-year follow up is 50.25%, 70% and 87.5% respectively. Five-year complete renal remission calculated by Kaplan-Meier analysis is 58.4%. Although without significant difference, there is trend of better survival with complete renal remission in group of nephrotic range proteinuria onset. There is no severe adverse effect.. This study supports the use of glucocorticoids plus immunosuppressive in addition to ACEI in IgA nephrology pediatric patients with proteinuria. We suggest proactive immunosuppressive treatment in IgA nephropathy in children. This is from a single center in China as may not same results in other population.

Topics: Angiotensin-Converting Enzyme Inhibitors; Biopsy; CD4-Positive T-Lymphocytes; Child; China; Cyclophosphamide; East Asian People; Female; Follow-Up Studies; Glomerulonephritis, IGA; Glucocorticoids; Hematuria; Humans; Immunosuppressive Agents; Intraocular Pressure; Kaplan-Meier Estimate; Kidney Diseases; Male; Mycophenolic Acid; Proteinuria; Retrospective Studies; Survival Analysis; Time Factors; Treatment Outcome

Children with severe Henoch-Schönlein purpura nephritis (HSPN) may progress to end-stage renal disease without appropriate treatment.. This study aimed to investigate the efficacy and safety of tacrolimus combined with glucocorticoids in the treatment of pediatric HSPN.. A total of 87 HSPN patients with urinary protein ≥ 0.75 g/24 h received standard of care, including angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and glucocorticoids. Patients were divided into three groups and additionally received tacrolimus (n = 30), cyclophosphamide (n = 31), or mycophenolate mofetil (MMF) (n = 26). We monitored outcome measures, including proteinuria, hematuria, and renal function and analyzed the efficacy and side effects in each group.. At 2-month follow-up, the overall efficacy was 93.3%, 83.9%, and 61.5% for tacrolimus, cyclophosphamide, and MMF, respectively (P < 0.05). Urinary protein significantly decreased for all groups. Urinary red blood cell counts significantly decreased for patients treated with tacrolimus (P < 0.001) and cyclophosphamide (P < 0.05), whereas no significant decrease was seen for those receiving MMF (P = 0.09). Although urine β2-microglobulin significantly decreased following 2 months of treatment with all medications, efficacy was greater with tacrolimus than with cyclophosphamide and MMF (P < 0.001). Major adverse events were respiratory and urinary infections, with MMF having the highest infection rate. The cyclophosphamide group also experienced additional adverse events, including arrhythmia, hemorrhagic cystitis, leukocytosis, thrombocytopenia, and hyperglycemia.. These results indicate that tacrolimus is more effective at reducing proteinuria and hematuria and improving renal function, with relatively milder side effects, in the treatment of pediatric HSPN.. ChiCTR2200055323, retrospectively registered on January 7, 2022.

Topics: Child; Cyclophosphamide; Glucocorticoids; Hematuria; Humans; IgA Vasculitis; Immunosuppressive Agents; Mycophenolic Acid; Nephritis; Proteinuria; Tacrolimus

Lupus nephritis requires antinuclear antibodies as classification criteria. There is a group of patients with nephrotic syndrome and conclusive histopathological findings for lupus nephritis, without classification criteria for systemic lupus erythematosus (SLE) or extrarenal manifestations. These groups of patients have been described as "lupus-like" nephritis or "renal-limited lupus nephritis".. Renal biopsy with histopathological evaluation with "full-house" immune-reactants in patients with negative antinuclear antibodies.. We report four cases with nephrotic syndrome and one with hematuria-proteinuria syndrome: two with impaired glomerular filtration rate and three with preserved renal function; urinary sediment with hematuria without dysmorphia and without extrarenal manifestations for autoimmune disease, negative antinuclear antibodies (ANA) and anti-double stranded DNA (anti-dsDNA); normal C3 and C4 complement levels. Renal biopsy in all cases was consistent for lupus nephritis class V. All patients received treatment as lupus nephritis protocol; only one case received induction with cyclophosphamide and methylprednisolone boluses, the rest received mycophenolic acid and prednisone as induction and maintenance. Two of the cases induced with mycophenolic acid relapsed, requiring cyclophosphamide for 6 months, achieving complete remission. All patients received renin-angiotensin-aldosterone system blockade and hydroxychloroquine. At follow-up, 4 cases still have negative antibodies and are without extrarenal manifestations for SLE classification criteria. The other case, during pregnancy several years after initial diagnosis, had preeclampsia with nephrotic proteinuria and a new determination of positive ANA and anti-dsDNA antibodies, complement levels below normal limits.. The follow-up of patients with membranous glomerulopathy must be close; lupus like nephritis may be the first manifestation of the disease.

Topics: Antibodies, Antinuclear; Complement System Proteins; Cyclophosphamide; Female; Glomerulonephritis, Membranous; Hematuria; Hospitals; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis; Mexico; Mycophenolic Acid; Pregnancy; Proteinuria

Since there are only a few reports on pediatric systemic lupus erythematosus (pSLE) in Chinese populations, therefore we retrospectively report the clinical and immunological features as well as renal outcome in Chinese pSLE.. Patients diagnosed with pSLE at Shanghai Children's Medical Center between 2001 and 2016 were evaluated and clinical data were retrospectively collected.. A total of 102 pSLE patients were analyzed. Renal disorder including proteinuria (81.37%) and hematuria (65.69%) were most commonly identified. Class IV was the most common finding on renal biopsy. In lupus nephritis (LN), 67.21%, 78.0%, 86.0% and 94.55% achieved complete remission within 6, 12, 18 and 24 months, respectively. Furthermore, 16.67% of LN patients suffered at least one renal flare. Antinuclear antibodies were detected in nearly all patients (97.62%), followed by anti-double-stranded DNA (anti-dsDNA) antibodies (70.0%) and anti-Sjögren's syndrome A (anti-SSA) antibodies (60.64%). Oral corticosteroid (93.14%) and mycophenolate mofetil (64.71%) was used in the majority of patients. Infection (32.35%) was the main side effect caused by the medications.. Our population-based pSLE cohort indicated that compared to other international cohorts, there was a higher prevalence of LN in Chinese pSLE. Proteinuria was the most frequent manifestation both at disease onset and during the entire clinical course. Class IV LN was the dominant renal pathological type. Nevertheless, there was a favorable renal remission rate and relatively low incidence of renal flare in our cohort. Apart from antinuclear antibodies and anti-dsDNA antibodies, anti-SSA antibodies were most frequently detected. Infection was the leading complication caused by the medications.

Topics: Adolescent; Adrenal Cortex Hormones; Age of Onset; Antibodies, Antinuclear; Biomarkers; Child; China; Female; Hematuria; Humans; Immunocompromised Host; Incidence; Lupus Erythematosus, Systemic; Lupus Nephritis; Male; Mycophenolic Acid; Opportunistic Infections; Prevalence; Proteinuria; Remission Induction; Retrospective Studies; Risk Factors; Time Factors; Treatment Outcome

The Henoch-Schönlein Purpura (HSP) orIgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae.. To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis.. Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants.. From the178 patients with IgA vasculitis,nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids.. Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Topics: Adolescent; Antirheumatic Agents; Azathioprine; Child; Child, Preschool; Cyclophosphamide; Female; Hematuria; Humans; IgA Vasculitis; Immunosuppressive Agents; Male; Mycophenolic Acid; Nephritis; Proteinuria; Retrospective Studies; Treatment Outcome

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up.

Topics: Adult; Complement C1q; Cyclophosphamide; Female; Glomerulonephritis, Membranoproliferative; Hematuria; Humans; Kidney Failure, Chronic; Mycophenolic Acid; Proteinuria; Rituximab; Syndrome; Urticaria; Vasculitis

Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren's syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients with autoimmune diseases.

Topics: Adrenal Cortex Hormones; Aged; Antibiotics, Antineoplastic; Autoimmune Diseases; Cryoglobulinemia; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative; Hashimoto Disease; Hematuria; Hepatitis, Autoimmune; Humans; Kidney; Methylprednisolone; Mycophenolic Acid; Nephritis, Interstitial; Pancytopenia; Renal Insufficiency; Sjogren's Syndrome; Treatment Outcome

To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity.. A case-control study.. Patients (n=49) were retrospectively included from Jinling Hospital in China if presenting with biopsy-proven ANCA-positive LN between 1985 and 2008. Clinicopathological characteristics and outcomes were analysed and compared with those of a control group (n=1279). We further compared treatment responses and outcomes of ANCA-positive LN patients based on the treatment issued.. The study included 40 women and 9 men (median age 33 years at biopsy): 38 with myeloperoxidase (MPO)-ANCA, 7 with proteinase 3. The characteristics of ANCA-positive LN were massive haematuria and advanced renal insufficiency. We observed a higher remission rate and better prognoses when using mycophenolate mofetil than when using cyclophosphamide as induction therapy.

Topics: Adult; Antibodies, Antineutrophil Cytoplasmic; Case-Control Studies; China; Cyclophosphamide; Female; Hematuria; Humans; Immunosuppressive Agents; Lupus Nephritis; Male; Mycophenolic Acid; Remission Induction; Renal Insufficiency; Retrospective Studies; Treatment Outcome

Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment.. Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively.. The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed.. KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.

Topics: Adolescent; Adult; Angiolymphoid Hyperplasia with Eosinophilia; Anti-Inflammatory Agents; China; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative; Glomerulonephritis, Membranous; Glomerulosclerosis, Focal Segmental; Hematuria; Humans; Hypertension; Immunosuppressive Agents; Isoxazoles; Leflunomide; Male; Middle Aged; Mycophenolic Acid; Phytotherapy; Plant Preparations; Prednisone; Prognosis; Proteinuria; Recurrence; Renal Insufficiency; Retrospective Studies; Tacrolimus; Tripterygium; Young Adult

The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied.. Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed.. The most common initial symptom of EGPA was asthma (57.1 %), followed by hemoptysis (21.4 %), gross hematuria (14.3 %), and arthritis (7.1 %). All patients had positive serum ANCA (anti-MPO in 12, anti-PR3 in 2). Elevated eosinophils (median 15 %, range 10-45 %) were found in all patients. The median serum IgE level was 463 g/L (range 200-1000 g/L). All patients presented with renal dysfunction, with a median SCr of 5.4 mg/dL (range 1.47-11 mg/dL), seven patients (50 %) required initial renal replacement therapy. Thirteen patients showed hematuria and proteinuria (median 1.1 g/24 h, range 0.5-7.8 g/24 h). Renal biopsy showed pauci-immune segmental necrotizing glomerulonephritis with crescents in 13 patients and acute interstitial nephritis in one patient. Twelve patients (85.7 %) showed renal interstitial eosinophil infiltration, among whom three had eosinophilic granuloma. Among seven patients (71.4 %) who required initial dialysis, 5 discontinued dialysis, one died, one received maintenance dialysis after glucocorticoids plus immunosuppressive for induction treatment. Twelve patients were followed up for a median of 43.5 months (range 6-83 months), during follow-up, two patients progressed to end-stage renal disease, nine had chronic kidney disease with eGFR < 60 mL/min, and two patients had normal eGFR.. Renal involvement in ANCA-positive EGPA could be severe and showed varied renal histology. Although intensive immunosuppressive therapy effectively improved the renal function, the long-term renal survival was poor. Early diagnosis and treatment are essential to improve long-term renal survival.

Topics: Adolescent; Adult; Aged; Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Blood Cell Count; China; Churg-Strauss Syndrome; Creatinine; Cyclophosphamide; Disease Progression; Drug Therapy, Combination; Eosinophils; Female; Glomerular Filtration Rate; Hematuria; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Male; Methylprednisolone; Middle Aged; Mycophenolic Acid; Prednisone; Proteinuria; Renal Dialysis; Renal Insufficiency, Chronic; Retrospective Studies; Time Factors; Young Adult

To retrospectively investigate clinico-pathological features and outcomes of patients with renal involvement in propylthiouracil (PTU)-associated antineutrophil cytoplasmic autoantibody (ANCA) vasculitis (PTU-AAV).. Clinico-pathological features and outcomes of 12 patients (female 11, average age 32.4 ± 13.8 years) who developed AAV after treatment with PTU were collected and analyzed. ANCA was detected by both immunofluorescence (IF) and enzyme linked immunosorbent assay (ELISA). All patients had renal biopsy.. Twelve patients received PTU for 2-264 months (median 42 months) when PTUAAV was diagnosed. All patients had positive serum P-ANCA, 11 of them were MPO-ANCA, 1 was MPO and PR3-ANCA double positive. All patients presented with hematuria and proteinuria, 5 of them had gross hematuria, urine protein was 1.9 ± 1.6 g/24 h, 7 of 12 (58.3%) patients had renal dysfunction, among them 3 needed initial renal replacement therapy. Renal biopsy showed pauci-immune segmental necrotizing crescentic glomerulonephritis in ten patients, segmental necrotizing glomerulonephritis superimposed on membranous nephropathy in two patients. All patients withdrew PTU and received steroid and immunosuppressive therapy. After a median follow-up of 42 months (range 21-86), 3 patients developed to ESRD, 7 patients entered complete renal remission. Serum ANCA turned negative only in 2 patients, 10 patients had persistent positive serum ANCA. Three patients relapsed with the elevation of serum ANCA level.. Renal damage of PTU-AAV could be pauci-immune necrotizing crescentic glomerulonephritis, and necrotizing glomerulonephritis coexisted with membranous nephropathy. Most patients had persistent positive serum ANCA and had a risk of relapse and progression to ESRD even after PTU withdrawal and immunosuppressive therapy.

Topics: Adolescent; Adult; Anti-Inflammatory Agents; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antithyroid Agents; Child; Cyclophosphamide; Disease Progression; Hematuria; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Glomerulus; Kidney Tubules; Male; Middle Aged; Mycophenolic Acid; Prednisone; Propylthiouracil; Proteinuria; Recurrence; Remission Induction; Renal Replacement Therapy; Retrospective Studies; Thyroid Diseases; Young Adult

Topics: Acute Kidney Injury; Adult; Blood Component Transfusion; Bone Marrow; Cell Lineage; Combined Modality Therapy; Darbepoetin alfa; Erythropoietin; Filgrastim; Granulocyte Colony-Stimulating Factor; Hematuria; Humans; Lupus Erythematosus, Systemic; Lupus Nephritis; Lymphohistiocytosis, Hemophagocytic; Male; Mycophenolic Acid; Myelodysplastic Syndromes; Pancytopenia; Pericarditis; Prednisone; Proteinuria; Recombinant Proteins

To describe the clinical characteristics, histopathologic features, and outcomes of patients in whom vasculitis developed in association with use of tumor necrosis factor-α (TNF-α) inhibitors.. This is a retrospective review of patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1, 1998, through March 31, 2011, with a diagnosis of vasculitis induced by anti-TNF-α therapy.. Of 8 patients with vasculitis associated with anti-TNF-α therapy (mean age, 48.5 years), 6 (75%) were female. Four (50%) had rheumatoid arthritis, 1 (13%) had Crohn disease, and 3 (38%) had ulcerative colitis. Five (63%) were treated with infliximab, 2 (25%) with etanercept, and 1 (13%) with adalimumab. The mean duration of treatment before development of vasculitis was 34.5 months. The skin was the predominant organ affected (5 patients [63%]), with the most common cutaneous lesion being palpable purpura (4 of 5 [80%]). Two organs involved in systemic vasculitis were the peripheral nervous system (4 patients [50%]) and kidney (1 patient [13%]). All cases of vasculitis were histopathologically confirmed. Seven of 8 patients improved with discontinuation of therapy (mean time to resolution, 6.9 months) and adjuvant treatment (all 8 received prednisone; another agent was also used in 7); rechallenge with anti-TNF-α therapy was not attempted in any patient. At last follow-up, no patients had experienced a recurrence of vasculitis after therapy discontinuation.. Cutaneous small-vessel vasculitis was the most common finding, but systemic vasculitis, including peripheral nerve and renal vasculitis, was also frequently observed.

Topics: Adalimumab; Adult; Aged; Anti-Inflammatory Agents; Antibodies, Monoclonal; Antibodies, Monoclonal, Humanized; Antibodies, Monoclonal, Murine-Derived; Arthritis, Rheumatoid; Azathioprine; Colitis, Ulcerative; Crohn Disease; Cyclophosphamide; Etanercept; Female; Glucocorticoids; Hematuria; Humans; Immunoglobulin G; Immunologic Factors; Infliximab; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Polyneuropathies; Prednisone; Proteinuria; Receptors, Tumor Necrosis Factor; Retrospective Studies; Rituximab; Tumor Necrosis Factor-alpha; Vasculitis; Young Adult

A 53-year-old man who underwent successful kidney transplantation for stage 5 chronic kidney disease presented to our clinic with intermittent painless gross hematuria. Urachal adenocarcinoma, stage III A by Sheldon system, was diagnosed after serial histopathologic and radiological studies. The patient was treated with extended partial cystectomy, en bloc resection of urachus and umbilicus, pelvic lymphadenectomy, and ileocystoplasty. There were no complications seen in this patient. Neither urachal adenocarcinoma recurrence, metastasis, nor de novo uroileal cancer developed during 48-month follow-up. His reconstructed bladder functioned efficiently, without compromising the transplanted kidney function. Our case demonstrated that conservative surgery and augmentation ileocystoplasty could be offered to kidney transplant recipients with localized urachal carcinoma.

Topics: Adenocarcinoma; Hematuria; Humans; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Neoplasms; Kidney Transplantation; Magnetic Resonance Imaging; Male; Middle Aged; Mycophenolic Acid; Neoplasm Staging; Prednisolone; Tacrolimus; Urachus

Long term cyclophosfamide combinated steroids therapy improves renal survival in patients with Proliferate Lupus Nephritis, with considerable toxic effects. In lately years MMF, a immune suppressor used in transplant, seems to be effective in selected cases of Lupus Nephritis.. We will describe six patients with Lupus Nephritis class IV and V (OMS Classification), that what different causes they must be treated with MMF like rescue drug. This stabilizes the renal function, controls LES activity and allows reductions or end of corticoids.. In tree cases we achievement total remission, two show partial remission and we had a fail because plaquetopenia and severe leucopenia with serious sepsis to give rise to neumopaty; this patient broke off the treatment. One presented Zoster Herpes that was treated with Aciclovir and temporary treatment break off.. MMF was effective to get remission in NL and maintain inactive LES, so it must to be considering as rescue therapy, or maintenance treatment after cyclosphosfamide induction. In patients where fertility is important must be considering like elective drug. Is necessary long time patients follow up to asses' effectiveness in renal survival, like cyclophosfamide.

Topics: Adult; Biopsy; Creatinine; Female; Hematuria; Humans; Immunosuppressive Agents; Lupus Nephritis; Middle Aged; Mycophenolic Acid; Proteinuria; Remission Induction; Salvage Therapy; Treatment Outcome