mycophenolic-acid and Conjunctivitis

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 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

To report two patients with sarcoidosis initially presenting with cicatrizing conjunctivitis.. Both patients with chronic conjunctivitis were referred for further management. The first patient had conjunctival granulomas, subepithelial fibrosis, and forniceal foreshortening. The second patient had extensive upper and lower conjunctival scarring with forniceal foreshortening and symblepharon formation of both eyes. Conjunctival biopsy specimens revealed noncaseating granulomas. Immunofluorescein studies were negative for immunoreactant deposition in the basement membrane. Because of further diagnostic evaluations, sarcoidosis was determined to be the final cause of the cicatrizing conjunctivitis.. Sarcoidosis should be included in the differential diagnosis of cicatrizing conjunctivitis. Recognition of the characteristic noncaseating granulomas in the conjunctival biopsy and initiation of the appropriate evaluations are essential in establishing the diagnosis and determining the extent of systemic involvement.

Topics: Administration, Oral; Administration, Topical; Adult; Biopsy; Chronic Disease; Cicatrix; Conjunctivitis; Cyclosporine; Diagnosis, Differential; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Male; Methotrexate; Middle Aged; Mycophenolic Acid; Sarcoidosis, Pulmonary; Tomography, X-Ray Computed

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