mycophenolic-acid and Pemphigoid--Benign-Mucous-Membrane

This review article summarizes the recent literature in regards to advancements in the diagnosis and management of mucous membrane pemphigoid (MMP) published in the English literature since May 2011.. MMP is an uncommon, subepithelial blistering conjunctivitis that is commonly associated with vision loss. Distinct subgroups of MMP have recently been identified using advanced immunohistochemical techniques. Antiepiligrin MMP has been described as a distinct entity with a relationship to certain solid organ malignancies. There is evidence that conventional immunomodulators such as mycophenolate mofetil and biologics such as rituximab are effective in halting disease progression. Unfortunately, the majority of patients at presentation to tertiary centers already have advanced disease. Novel regenerative techniques such as stem cell therapy as well as traditional surgical approaches such as keratoprosthesis might be required for the restoration of vision in patients with significant corneal scarring.. Despite advances in available immunosuppressive treatment regimens, significant ocular morbidity persists possibly secondary to difficulty in diagnosis. Additionally, absence of clinically detectable inflammation in a significant proportion of patients causes delay in the treatment and leads to progressive scarring. Surgical management of the complications of MMP remains challenging, with high failure rates over long-term follow-up.

Topics: Antibodies, Monoclonal, Murine-Derived; Conjunctivitis; Humans; Immunologic Factors; Mucous Membrane; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Rituximab

To describe the clinical features and course of 2 patients with autoimmune diseases and their experience with the Boston keratoprosthesis. To draw on general medical literature to try to better understand recurrent complications.. Retrospective review of 2 patients treated with Boston keratoprostheses. The clinical histories, examinations, and other diagnostics were reviewed. A literature review was performed.. The first patient presented with end-stage ocular disease secondary to toxic epidermal necrolysis (TENS). The second patient presented with end-stage ocular disease secondary to mucous membrane pemphigoid (MMP). Both patients underwent treatment with the Boston keratoprosthesis. Both patients suffered numerous corneal melts requiring multiple repeat implantations.. Patients with corneal blindness secondary to autoimmune disease often fare poorly with available surgical treatments. Study of existing literature on prosthetic device complications in autoimmune diseases may help uncover common mechanisms of tissue destruction to establish perioperative immunomodulatory regimens targeted to specific underlying diseases.

Topics: Adult; Alternaria; Anti-Inflammatory Agents; Antibodies, Monoclonal; Autoimmune Diseases; Blindness; Corneal Diseases; Female; Humans; Infliximab; Male; Middle Aged; Mycophenolic Acid; Mycoses; Pemphigoid, Benign Mucous Membrane; Prednisolone; Prostheses and Implants; Prosthesis Implantation; Stevens-Johnson Syndrome; Treatment Outcome; Visual Acuity

Immunosuppressive treatment has shown to be effective in various ocular inflammatory disorders. Factors limiting their use are the individual response and the rate of side effects. This report summarizes our knowledge about the use of mycophenolate mofetil (MMF) in the treatment of ocular cicatricial pemphigoid (OCP), uveitis, atopic keratoconjunctivitis (AKC), prevention of graft rejection after penetrating keratoplasty (PK) and scleritis. Controlled studies have been performed for prevention of graft rejection after PK, showing MMF as effective in the prevention of graft rejection as cyclosporine A. In experimental uveitis, MMF has been demonstrated to be highly effective in prevention of retinal destruction. A number of studies have now shown that MMF also seems effective in uveitis. There are also studies with smaller patient groups which point out the effectiveness of MMF in OCP, AKC, and scleritis. In most of the studies, the spectrum of side effects was small, compared to other immunosuppressives.

Topics: Eye Diseases; Graft Rejection; Humans; Immunosuppressive Agents; Keratoplasty, Penetrating; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane

Up to now ophthalmologists have only a small number of substances in their therapeutic armamentarium for the treatment of ocular immunological disorders. These are very potent agents, and any rapidly proliferating cells are extremely sensitive to such agents, thus the possibility of severe side effects is great. Mycophenolatemofetil (MMF) has been shown to be effective in preventing acute graft rejection following clinical renal and cardiac transplantation with a low risk of adverse events. Likewise MMF was effective in the treatment of experimental immunological disorders. A review of literature was performed in Medline.. We report on the effective use of MMF in a patient with ocular cicatricial pemphigoid, the switch from Cyclosporin A (CSA) therapy to MMF therapy following high-risk keratoplasty due to CSA allergy and about the combination therapy (CSA/MMF) in a patient following high risk keratoplasty, in whom CSA monotherapy was not sufficient to prevent allograft rejection.. In these three cases MMF has been shown to be a safe and effective agent for the treatment of ocular immunological disorders. Whether these beneficial casuistic experiences will hold true will be depended on the outcome of longterm studies underway.

Topics: Corneal Transplantation; Cyclosporine; Drug Hypersensitivity; Eye Diseases; Female; Humans; Immunosuppressive Agents; Male; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane

The severity of cicatricial pemphigoid (CP) varies. First-intent treatment of mild or moderate cases is dapsone. In life or sight-threatening cases, intravenous cyclophosphamide pulses are efficient but may have digestive side effects and imply repeated hospitalizations. Mycophenolate mofetil (MMF) is an oral and well tolerated immunosuppressant agent which has proved its efficacy in pemphigus and some bullous pemphigoid. In CP, encouraging case reports have been previously published. We report herein a retrospective study about 14 patients who have received MMF since 2000.. There were 5 men and 9 women, with a mean age of 69 years. MMF was introduced in 3 different clinical situations: immediately in relay to cyclophosphamide in 7 patients with severe CP (group I); in case of a mild-severe relapse at distance from with dranal of cyclophosphamide in 3 patients (group II); as first-intent immunosuppressant agent in 4 patients whose disease was not under control with high-dose dapsone, but not life - or sight-threatening (group III). In all these patients, the disease was invalidating and not controlled by dapsone +/- sulfasalazine, but did not threaten life or sight. The aim was to achieve satisfying control of the disease with an oral and well tolerated immunosuppressant agent, and to maintain good quality of life. The dose of MMF was 1.5 or 2 g per day. The criteria of MMF efficacy was the healing of previous lesions and the absence of new progressive lesions.. MMF was efficient in obtaining or maintaining a good control of the disease in 10/14 patients, as long as the underlying treatment with dapsone (2 mg/kg/d) was maintained. In 7/10 cases, it was possible to decrease the dapsone dose in order to improve hematological tolerance. In the 3 other cases, a relapse occurred when the dose of dapsone was decreased. MMF was inefficient in controling the disease in 4/14 patients (29 p. 100). Clinical and biological tolerance of MMF was good in 13/14 patients.. In this series, MMF was proposed to heterogenous patients, who presented at that time a mild-moderate disease and for whom we wanted in improve the quality of life. MMF seems to be an interesting drug, capable of obtaining or maintaining satisfactory control of the disease and permitting the decrease of dapsone doses in some mild-severe CP. However MMF must not replace cyclophosphamide in severe sight or life-threatening forms of CP.

Topics: Administration, Oral; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Dapsone; Dermatologic Agents; Female; Humans; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Quality of Life; Retrospective Studies; Treatment Outcome

Ocular cicatrical pemphigoid can lead to severe structural damage or loss of vision at worst. Longterm therapy with dapsone or systemic immunosuppressive therapy, e.g. with cyclophosphamide is often inevitable. Immunosuppression may cause severe side effects in some patients.. Data are presented on 5 patients with ocular cicatrical pemphigoid who were treated with mycophenolate mofetil 2 g daily. Criterion of effectiveness was the clinical course of the condition defined as nonprogression of the morphologic alterations. Patients were initially examined and interviewed routinely every four weeks for the first four months, then every eight weeks. Patients were asked about side effects and underwent monthly blood checks.. All patients were followed for at least 12 months. Mycophenolate mofetil proved to be effective with respect to the clinical course in 9 out of 10 eyes. All patients showed regression of inflammatory conjunctival alteration and improvement of their complaints. In one eye the inflammatory process restarted after surgery due to excessive symblephara had been performed. Gastrointestinal side effects were reported in the initial phase, e.g. lack of appetite, nausea and mild diarrhoea.. Mycophenolate mofetil proved to be an effective immunosuppressant for the treatment of ocular cicatrical pemphigoid. Namely side effects were less severe and frequent compared to those known from other currently administered immunosuppressants. Longterm results and larger case numbers are needed to sustain these early results.

Topics: Adult; Aged; Aged, 80 and over; Cross-Over Studies; Female; Humans; Immunosuppressive Agents; Keratoconjunctivitis Sicca; Male; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Treatment Outcome; Visual Acuity

To evaluate the demographic data, ocular and systemic findings, clinical management, and outcomes of patients with ocular cicatricial pemphigoid (OCP).. The medical records of 11 patients diagnosed as having OCP in the ophthalmology department of Ege University between 2008 and 2021 were evaluated retrospectively.. The patients' mean follow-up time was 14±5.76 months. All eyes (100%) had conjunctival involvement and 18 (81.81%) had corneal involvement. According to the Tauber staging system, 7 (31.81%), 8 (36.36%), and 7 (31.81%) of the eyes were stage 2, 3, and 4, respectively. The diagnosis was confirmed in 6 (66.66%) of 9 patients who underwent biopsy. Amniotic membrane transplantation was performed in 7 eyes, entropion surgery in 2 eyes, and electrocauterization for trichiasis in 5 eyes. Systemic involvement was observed in 45.45% (5/11) of patients, most commonly oral mucosal involvement (18.18%). Review of medical records showed that alkylating agents, steroids, and dapsone were used in patients treated before 2020. Mycophenolate mofetil was preferred to be used in combination with corticosteroids. Although treatment responses before mycophenolate mofetil usage could not be evaluated well because of loss to follow-up, 4 (66.66%) of 6 patients who received steroid treatment combined with mycophenolate mofetil showed partial or complete clinical remission. No serious side effects and drug withdrawal were observed.. OCP is a sight-threatening autoimmune disease that affects older adults. Although positive biopsy results are valuable for diagnosis, negative results do not exclude the diagnosis. The main treatment is systemic immunosuppressives. Disease activity can be suppressed, especially with early initiation of drug therapy. These patients require a multidisciplinary approach. Especially in the presence of isolated ocular findings, ophthalmologists should be able to make the decision to start immunosuppressive treatment, and systemic treatment should not be delayed.

Topics: Aged; Conjunctiva; Humans; Immunosuppressive Agents; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Retrospective Studies

Topics: Eye; Humans; Immunosuppressive Agents; Mucous Membrane; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous

To characterize the clinical features of patients with direct immunofluorescence (DIF)-negative mucous membrane pemphigoid (MMP).. Retrospective case series.. Thirty-six patients who underwent a conjunctival biopsy for suspected MMP were included. Demographic and clinical information was collected. Main outcome measures included visual acuity, Foster stages, presence of extraocular involvement, history of autoimmune disease, and durations of follow-up.. Thirty-two patients had a negative DIF. Of those, 2 had a positive DIF on repeat biopsy. Eleven showed progression of conjunctival scarring during a median follow-up of 42 months (range, 8-100 months) and were diagnosed with biopsy-negative MMP. Another 11 patients with a median follow-up of 54 months (range, 15-138 months) were diagnosed with cicatrizing conjunctivitis of other causes. The median visual acuity of patients with biopsy-negative MMP at presentation was significantly lower compared to patients with cicatrizing conjunctivitis of other causes (20/400 vs 20/40, P = .02). Conjunctival scarring score at presentation in both biopsy-positive and biopsy-negative MMP groups was significantly higher compared to patients with cicatrizing conjunctivitis of other causes (median Foster stage, 3 vs 1, P = .009; and 3 vs 1, P = .01, respectively).. Patients with progressive cicatrizing conjunctivitis likely have MMP in the absence of alternate diagnoses. Our findings emphasize that suspicion for MMP must remain high for patients who have Foster stage 3 conjunctival scarring on presentation or worsening of scarring during follow-up, even in the setting of negative DIF.

Topics: Adult; Aged; Aged, 80 and over; Antibiotics, Antineoplastic; Autoimmune Diseases; Biopsy; Cicatrix; Conjunctivitis; Cyclophosphamide; Disease Progression; Female; Fluorescent Antibody Technique, Direct; Follow-Up Studies; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Retrospective Studies; Staining and Labeling; Visual Acuity

This is a report of a case with mucous membrane pemphigoid (MMP) with severe eye involvement and concurrent COVID-19 treated successfully using simultaneous high dose intravenous immunoglobulin (IVIg) and anti-viral treatment as hydroxychloroquine, lopinavir/ritonavir, and ribavirin. He had finished a 2-g cycle of rituximab (RTX) in late January. He was receiving mycophenolate mofetil (MMF) for one month and 30 mg prednisolone for three months until his hospitalization. Prednisolone was tapered to 15 mg when current COVID-19 was suspected, considering his recent cough, dyspnea, and fever.

Topics: Adult; Anti-Bacterial Agents; Antiviral Agents; Betacoronavirus; Coronavirus Infections; COVID-19; COVID-19 Drug Treatment; Deprescriptions; Diabetes Mellitus, Type 2; Drug Combinations; Drug Therapy, Combination; Humans; Hypertension; Immunoglobulins, Intravenous; Immunologic Factors; Iran; Lopinavir; Male; Mycophenolic Acid; Oseltamivir; Pandemics; Pemphigoid, Benign Mucous Membrane; Pneumonia, Viral; Prednisolone; Ritonavir; Rituximab; SARS-CoV-2; Tomography, Spiral Computed

Topics: Adrenal Cortex Hormones; Dapsone; Dysphonia; Humans; Immunologic Factors; Laryngeal Diseases; Laryngocele; Laryngoscopy; Male; Middle Aged; Mycophenolic Acid; Nasopharyngeal Diseases; Pemphigoid, Benign Mucous Membrane; Pharyngitis; Rituximab; Tomography, X-Ray Computed

Mucous membrane pemphigoid (MMP) encompasses a group of autoantibody-mediated, subepithelial blistering diseases, which primarily affect mucosal surfaces including oral, ocular, skin, genital, nasopharyngeal and oesophageal sites. We present a first description of laryngoceles as a manifestation of mucous membrane pemphigoid resulting in dynamic airway closure. Mucosal injury at other sites had previously resulted in pathergy and localised cicatrisation. We discuss successful combined medical and transcutaneous surgical intervention designed to avoid tracheostomy and minimise iatrogenic laryngeal cicatrisation.

Topics: Airway Obstruction; Antibiotics, Antineoplastic; Humans; Laryngocele; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Rituximab

To evaluate whether conjunctival biopsy findings in patients with ocular mucous membrane pemphigoid (MMP) persist as positive or revert to negative following treatment with immunomodulatory therapy (IMT).. Patients with biopsy-proven MMP were treated with IMT for at least 2 years before undergoing repeat conjunctival biopsy for immunofluorescence microscopy. Their records were reviewed and findings evaluated to ascertain which patients' biopsies showed antibody deposition on the conjunctival basement membrane.. Following 2 years of IMT, conjunctival biopsies showed persistent antibody deposition in two patients, and were negative in four patients.. Conjunctival biopsies in patients with ocular MMP may show reversion to inactive disease following IMT. Post-treatment biopsy might be clinically useful as a means of evaluating the efficacy of therapy in this chronic disease.

Topics: Aged; Aged, 80 and over; Azathioprine; Biopsy; Conjunctiva; Conjunctival Diseases; Cyclophosphamide; Female; Follow-Up Studies; Humans; Immunomodulation; Immunosuppressive Agents; Male; Microscopy, Fluorescence; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane

Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up.

Topics: Airway Obstruction; Antibiotics, Antineoplastic; Bronchoscopy; Female; Humans; Mucous Membrane; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Respiratory Distress Syndrome; Trachea; Tracheostomy; Treatment Outcome; Young Adult

Ocular cicatricial pemphigoid (OCP) is rare, severe, sight threatening autoimmune disease of the conjunctiva, which affects elderly patients, more often women.. To evaluate the success rate of stabilisation of ocular findings in patients with OCP.. Retrospective study of patients from Centre of Conjunctival and Corneal Diseases at Department of Ophthalmology, General University Hospital and 1st Medical Faculty of Charles University in Prague in 1992-2013 was performed. Frequency of OCP clinical stages, visual acuity (VA), disease activity and ocular complications of referred patients were monitored. Moreover, type of immunosuppressive (IS) therapy, the number of relapses of the disease and progress of OCP clinical stages were evaluated. Especially, we evaluated effects and side effects of mycophenolate mofetil (MM) therapy. In addition to that, type and frequency of ocular surgery that was carried out to the patients before and after the referral were recorded. Furthermore, we evaluated percentage of patients with mucous membranous pemphigoid (MMP). Also, the positive yield of diagnostic methods was assessed.. The OCP was diagnosed and monitored in 51 patients (21 men and 30 women) during 21 years, the average age on the day of diagnosis was 68,4 years, the average period of observation was 57 months. 55 % of eyes were referred to our department at clinical stage 3, 27 % at stage 4. VA was maintained in 76 % of eyes, improved in 5 % of eyes and in 19 % of eyes deteriorated. Activity of OCP was detected during the first examination in 96 % of patients, the most common complications at that time was corneal ulcer or perforation. Patients were treated by immunosuppressive therapy, most often in combination: corticosteroids (47 patients), azathioprine (28 patients), cyclophosphamide (25 patients), MM (16 patients), sulphasalazine (5 patients), dapsone (5 patients). We ascertained relapses in 40 % of patients. The progression to the next stage of OCP were found in 7 eyes (6,9 %) and 95 eyes (93,1 %) remained stable. Activity of disease was well controlled in 11 patients out of 16 (69 %) by MM, IS therapy of remain 5 patients (31 %) had to be changed. Side effects of MM such as lymphopenia were present in 1 patient. Before OCP was diagnosed, patients underwent cataract surgery with the intraocular lens implantation, cryoepilation of eyelashes and eyelid plastic surgery, especially entropion. The most common indicated surgery in our clinic was amniotic membrane transplantation and retro position of muscular cutaneous leaf. 31 % of patients were diagnosed with MMP. Positive results of conjunctival biopsy were detected in 48 % from 42 examined samples and 22 % from 32 examined samples had positive results of indirect immunofluorescence (anti-desmosoms).. OCP diagnosis is established on the basis of patient´s ophthalmic history and clinical findings. Positive results of direct and indirect immunofluorescence support the diagnosis. Activity of the disease and progression of OCP is effectively suppressed by systemic immunosuppressive therapy (for example MM), mainly if started at early stage of the disease.. ocular cicatricial pemphigoid, immunosuppressive therapy, direct and indirect immunofluorescence, mycophenolate mofetil.

Topics: Aged; Aged, 80 and over; Conjunctival Diseases; Enzyme Inhibitors; Female; Fluorescent Antibody Technique, Direct; Fluorescent Antibody Technique, Indirect; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Recurrence; Retrospective Studies; Visual Acuity

To describe the outcomes and medical management necessary to achieve successful lid surgery in patients with biopsy-confirmed and presumed mucous membrane pemphigoid.. Retrospective, interventional case series.. We included patients with positive biopsy results and cases with a typical clinical active bilateral presentation with negative biopsy results but classic features. We identified 11 operated eyes of 7 patients with lid malposition resulting from mucous membrane pemphigoid, particularly cicatricial entropion, that required surgical correction. Complete ophthalmologic history and examination were performed. The main outcome measures were control of ocular inflammation, progression of disease, and surgical success.. A bandage lens was used in 8 (72.7%) eyes to protect the cornea while immunosuppression and control of disease activity were achieved. Control of ocular inflammation before lid surgery was achieved in all cases. Immunosuppressive treatment before lid surgery was used in all cases for a mean of 15.1 months (range, 8.2 to 33.1 months) and after surgery for a mean of 6.6 months (range, 3.0 to 11.2 months). The oral immunosuppressive drugs used were mycophenolate and cyclophosphamide. Prednisone was used concomitantly in 4 (57%) patients. Full surgical success was achieved in all patients, with 1 patient requiring a second intervention because of residual disease. The mean postoperative follow-up period was 20.8 months (range, 6.0 to 30.5 months).. Successful entropion repair in patients with mucous membrane pemphigoid can be achieved if control of inflammation is attained before the procedure. Ocular surface protection while achieving disease control is essential in the management of these patients.

Topics: Administration, Oral; Aged; Aged, 80 and over; Biopsy; Cyclophosphamide; Drug Therapy, Combination; Entropion; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Ophthalmologic Surgical Procedures; Pemphigoid, Benign Mucous Membrane; Retrospective Studies; Suture Techniques; Visual Acuity

Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined.. A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months.. The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil.. Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone.

Topics: Aged; Autoantibodies; Autoantigens; Clobetasol; Dapsone; Drug Resistance; Humans; Immunoglobulin G; Immunosuppressive Agents; Laminin; Male; Methotrexate; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Pemphigoid, Bullous; Prednisolone; Remission Induction; Skin

To evaluate the clinical outcomes of mycophenolate mofetil (MMF) treatment of mucous membrane pemphigoid (MMP).. This is a retrospective analysis of consecutive patients with clinical MMP seen in the Ocular Surface Disease Clinic at the Wills Eye Institute, between January 1, 2004, and December 31, 2010, treated with MMF. The main outcomes measured were control of inflammation and discontinuation of MMF.. A total of 23 MMP patients taking MMF were identified. The median age of the MMF-treated patients was 77.0 years. Eleven of the 23 patients (47.8%) had biopsy-proven MMP. All patients were at least Foster grading system stage 2, with most stage 3 or 4. Eight patients (34.8%) failed previous treatments with dapsone, methotrexate, prednisone, azathioprine, cyclophosphamide, or 6-mercaptopurine. The average duration of MMF treatment was 23.32 ± 33.17 months (range 1-124.83 months, median 7.4 months). Of the 23 patients with MMP, control of inflammation was achieved with MMF within 3 months for 56.5% [95% confidence interval (CI) 54.5-59.6], within 6 months for 69.6% (95% CI 65.2-76.6), and within 12 months for 82.6% (95% CI 75.3-92.4) of the patients. Nineteen patients (82.4%) achieved control of inflammation, with 16 of the 19 (84.2%) achieving control of inflammation with MMF as monotherapy. Fifteen patients were treated with MMF as initial therapy. Twenty-one percent of patients (5 of 23) were taken off MMF for failure of inflammatory control (4) or an allergic reaction (1).. Treatment of MMP with MMF in this uncontrolled case series resulted in control of inflammation in the majority of patients with minimal side effects. Our data support consideration of MMF as an initial treatment option for active ocular MMP.

Topics: Aged; Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Conjunctiva; Conjunctivitis; Female; Humans; Immunosuppressive Agents; Male; Mucous Membrane; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Retrospective Studies; Treatment Outcome; Visual Acuity

Topics: Aged; Basement Membrane; Biopsy; Conjunctiva; Conjunctivitis; Diagnosis, Differential; Drug Therapy, Combination; Female; Fluorescent Antibody Technique, Direct; Glucocorticoids; Humans; Immunoglobulin A; Immunoglobulin G; Immunosuppressive Agents; Mouth Mucosa; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Prednisolone

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disorder characterized by inflammation, blistering, and scarring and predominantly occurring at mucous membranes. Successful treatment can be challenging, and uncontrolled disease may result in significant morbidity with scarring of the conjunctiva and oropharynx leading to blindness and dysphagia, respectively. We report safe successful treatment of 6 patients with significant MMP-related oral inflammation with the use of a previously unreported combination of mycophenolate mofetil, dapsone, and prednisolone given at relatively low doses. We propose that this combination of treatments should be investigated further.

Topics: Adult; Aged; Aged, 80 and over; Anti-Infective Agents; Dapsone; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Prednisolone; Treatment Outcome

To assess the long-term efficacy and tolerability of mycophenolate mofetil (MMF) in the therapy of ocular mucous membrane pemphigoid (OcMMP).. Ten patients (19 eyes) with OcMMP treated with MMF, who had a follow-up of at least 4 years, were retrospectively analyzed. Main outcome measures were inflammatory control, progression of cicatrization, and side effects.. Control of inflammation was achieved in 11 eyes (58%) of 6 patients (rate: 0.11/PY). Mild inflammatory activity was observed in 8 eyes (42%) of 4 patients (rate: 0.07/PY). Progression of conjunctival cicatrization was prevented in 47% (9/19) of the eyes. Mild progression of cicatrization was found in 42% (8/19) of the eyes. Conjunctival cicatrization had progressed to stage IV in 1 patient (11%). Treatment-related side effects occurred in 7 patients (rate: 0.12/PY).. MMF is an effective agent for treatment of OcMMP. However, the drug cannot always prevent disease progression.

Topics: Aged; Cicatrix; Disease Progression; Female; Humans; Immunosuppressive Agents; Inflammation; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Retrospective Studies; Treatment Outcome

To evaluate mycophenolate mofetil as a single noncorticosteroid immunosuppressive treatment for noninfectious ocular inflammatory diseases.. Retrospective cohort study.. Characteristics of patients with noninfectious ocular inflammation treated with mycophenolate mofetil at 4 subspecialty clinics from 1995 to 2007 were abstracted by expert reviewers in a standardized chart review of every eye at every visit. Main outcomes measured were control of inflammation, corticosteroid-sparing effects, and discontinuation of mycophenolate mofetil (including the reasons for discontinuation). Survival analysis was used to estimate the incidence of outcomes, and to identify risk factors for each.. Among 236 patients (397 eyes) treated with mycophenolate mofetil monotherapy, 20.3%, 11.9%, and 39.8% had anterior uveitis, intermediate uveitis, and posterior uveitis or panuveitis respectively; 14% had scleritis; 7.6% had mucous membrane pemphigoid; and 6.4% had other ocular inflammatory diseases. By Kaplan-Meier estimation, complete control of inflammation--sustained over consecutive visits spanning at least 28 days--was achieved in 53% and 73% of patients within 6 months and 1 year respectively. Systemic corticosteroid dosage was reduced to 10 mg of prednisone or less, while maintaining sustained control of inflammation, in 41% and 55% of patients in 6 months and 1 year respectively. Twelve percent of patients discontinued mycophenolate mofetil within the first year because of side effects of therapy.. Given sufficient time, mycophenolate mofetil was effective in managing ocular inflammation in approximately half of the treated patients. Treatment-limiting side effects were observed in 12% of patients and typically were reversible.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Child; Female; Follow-Up Studies; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Prednisone; Retrospective Studies; Risk Factors; Scleritis; Treatment Outcome; Uveitis; Visual Acuity

Mucous membrane pemphigoid belongs to the family of sub-epidermal bullous diseases and is clinically characterized by mucosal involvement leading to fibrosis and scarring. Cutaneous involvement is unfrequent and oral mucosa and conjunctivas are the most frequently involves mucosa. Treatment relies on anti-inflammatory and immunosuppressive drugs. The main goal is the limitation of the fibrosing potential of the autoimmune deposits within various mucous membranes.

Topics: Algorithms; Anti-Inflammatory Agents; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Autoimmune Diseases; Cyclophosphamide; Dapsone; Decision Trees; Diagnosis, Differential; Enzyme-Linked Immunosorbent Assay; Fluorescent Antibody Technique; France; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Rituximab; Sulfasalazine; Tumor Necrosis Factor-alpha

Topics: Azathioprine; Conjunctival Diseases; Cyclophosphamide; Dapsone; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Sulfapyridine; Treatment Outcome; Visual Acuity

To evaluate the effectiveness and toxicity of a stepladder immunosuppression strategy, including the use of mycophenolate mofetil and combination therapy, in the treatment of ocular mucous membrane pemphigoid.. Retrospective, noncomparative, interventional case series.. Two hundred twenty-three eyes of 115 patients.. Patients with a diagnosis of ocular mucous membrane pemphigoid commencing immunosuppression between January 1994 and July 2005 were identified. A treatment episode was defined by the use of a particular therapy or combination of therapies.. For each treatment episode, success of immunosuppressive therapy in controlling ocular inflammation was graded as a success (S), qualified success (QS), or failure (F). Initial and final visual acuities (VAs), stage of cicatrization (Foster, Mondino), grade of conjunctival inflammation, and side effects were recorded.. In 70% (80/115) of patients, inflammation was controlled by the end of the study. At least 6 months remission off treatment occurred in 16 patients (14%). Of the 388 treatment episodes, 50% were classified as S; 27%, QS; and 23%, F. The most successful therapies were based on cyclophosphamide (S, 69%; QS, 21%; F, 10%), followed by mycophenolate (S, 59%; QS, 22%; F, 19%), azathioprine (S, 47%; QS, 24%; F, 29%), dapsone (S, 47%; QS, 30%; F, 23%), and sulfapyridine (S, 38%; QS, 27%; F, 35%). Combination sulfa-steroid-myelosuppressive agent therapy increased the response from 73% with single-agent therapy to 87%. Side effects were the reason for 29% of changes in therapy. These were most prominent with azathioprine (40%) and least with mycophenolate (15%). Initial best-corrected VA (BCVA) was 6/60 or less in 17% (37/223) of eyes, pemphigoid being the cause in 13% (29/223). Final BCVA was 6/60 or less in 34% (76/223) of eyes, pemphigoid being the cause in 26% (57/223). By the end of the study, Mondino stage cicatrization had progressed in 41% (92/223) of eyes and 53% (61/115) of patients.. Mycophenolate mofetil seems to be an effective and well-tolerated immunosuppressant for moderately active ocular mucous membrane pemphigoid. Combination sulfa-steroid-myelosuppressive agent therapy in a stepladder regimen is a useful strategy to improve disease control. Cicatrization and VA may still progress and worsen despite adequate control of inflammation.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Azathioprine; Conjunctival Diseases; Cyclophosphamide; Dapsone; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Retrospective Studies; Sulfapyridine; Treatment Outcome; Visual Acuity

Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Scarring can result in severe structural changes to the vulva that may mimic the findings of other inflammatory dermatologic disorders of the vulva, including lichen sclerosus and lichen planus.. A 58-year-old woman presented with vulvar erosions, esophagitis, and laryngeal blisters. The clinical picture and the histopathology of a vulvar biopsy were suggestive of erosive lichen planus. Direct immunofluorescence, however, revealed findings diagnostic of mucous membrane pemphigoid.. This case illustrates the importance of examining extragenital mucosal surfaces of any woman presenting with vulvar lesions. In addition, it demonstrates the importance of vulvar biopsy and the usefulness of direct immunofluorescence to differentiate between conditions with similar clinical and histopathologic changes.

Topics: Biopsy, Needle; Drug Therapy, Combination; Female; Fluorescent Antibody Technique, Direct; Follow-Up Studies; Humans; Immunohistochemistry; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane; Prednisone; Severity of Illness Index; Treatment Outcome; Vulvar Diseases

To evaluate treatment outcomes with mycophenolate mofetil in patients with inflammatory eye disease.. Retrospective case series.. Eighty-four consecutive patients with inflammatory eye disease treated with mycophenolate mofetil at an academic referral center.. Medical records were reviewed for treatment with mycophenolate mofetil. Dose of mycophenolate mofetil, response to therapy, dose of prednisone, use of other immunosuppressive drugs, and side effects associated with the use of mycophenolate mofetil were recorded.. Ability to control ocular inflammation with mycophenolate mofetil and to taper prednisone to < or =10 mg daily, and incidence of treatment-related side effects.. Of the 84 patients treated with mycophenolate mofetil, 61% had uveitis, 17% had scleritis, 11% had mucous membrane pemphigoid, and 11% had orbital or other inflammatory disease. Forty-three percent of patients treated with mycophenolate mofetil had been treated with at least one other immunosuppressive drug previously. The median dose of prednisone at the start of mycophenolate mofetil therapy was 40 mg, and 82% of the patients were considered a treatment success, as judged by the ability to control the inflammation and taper prednisone to < or =10 mg daily. Median time to treatment success was 3.5 months. Mycophenolate mofetil therapy was discontinued due to insufficient efficacy at a rate of 0.10 per person-year (PY) and due to side effects at a rate of 0.08/PY. The most frequent side effect was gastrointestinal upset, with a rate of 0.19/PY.. These data suggest that mycophenolate mofetil may be an effective corticosteroid-sparing agent in the treatment of inflammatory eye disease with a manageable side effect profile.

Topics: Adolescent; Adult; Aged; Child; Conjunctival Diseases; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Orbital Pseudotumor; Pemphigoid, Benign Mucous Membrane; Prednisone; Retrospective Studies; Scleritis; Treatment Outcome; Uveitis

Topics: Humans; Immunosuppressive Agents; Keratoconjunctivitis; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane

Cicatricial pemphigoid (CP) is a rare autoimmune bullous disease that affects the skin and mucous membranes. It commonly ends by serious complications such as blindness, stenosis, and stricture formation and is difficult to treat. Mycophenolate mofetil has been reported to be effective in the treatment of pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid either as monotherapy or as a steroid-sparing agent.. Our purpose was to evaluate the effectiveness of mycophenolate mofetil as a steroid-sparing agent in treating patients with CP.. Three patients with CP were treated with mycophenolate mofetil and prednisolone.. All 3 patients responded very well to the therapy. None of them showed relapse of the disease for a follow-up period of 6 to 14 months after complete cessation of mycophenolate mofetil and prednisolone. No side effects were seen.. Mycophenolate mofetil appears to be a safe and effective steroid-sparing agent in the treatment of CP.

Topics: Anti-Inflammatory Agents, Non-Steroidal; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mycophenolic Acid; Pemphigoid, Benign Mucous Membrane