- Ophthalmology. 2002 Jan;109(1):111-8.
Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., Boston, MA 02114, USA.
PURPOSE: To determine the clinical outcome of patients with ocular-cicatricial pemphigoid (OCP) and the influence of systemic treatment on clinical progression. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Sixty-one patients with biopsy-proven OCP. METHODS: Patients with documented disease progression treated with chemotherapy and/or corticosteroids were followed between 1985 and 2000. The parameters evaluated were ocular stage at presentation, visual acuity, ocular complications, disease progression, control of ocular inflammation, and presence of extraocular involvement. Systemic treatment and related side effects were analyzed. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, extraocular involvement, disease progression, clinical outcome, systemic treatment, and related side effects. RESULTS: Sixty-one patients (32 female; 29 male) with a mean age of 67 years were studied. Extraocular involvement was present in 50% of patients. Sixty percent of eyes were initially seen with stage III (advanced cicatrizing) disease at first evaluation. Seven percent of involved eyes at first visit and 21% at the end of follow-up were legally blind. The most common ocular complications encountered were dry eye, corneal abnormalities, and glaucoma. Dapsone was the most commonly used drug (51 patients), followed by methotrexate (24 patients), azathioprine (23 patients), and cyclophosphamide (15 patients); prednisone, always given as adjunctive treatment, was used in 17 patients. Control of ocular inflammation (total or partial) was achieved in 90% of patients, but 46% of them needed continuation of systemic treatment to avoid disease recurrences, and 10% progressed despite different drugs used. Two agents were required in 32% of cases to control disease activity. The most common treatment-related side effects were hematologic complications (n = 34) followed by gastrointestinal (n = 17), cardiovascular (n = 15), and urinary complications (n = 11). Dapsone was responsible for the greatest number of side effects (n = 43); methotrexate caused the least trouble (n = 6). Corticosteroid-related complications (n = 34) were mostly cardiovascular and endocrinologic. CONCLUSIONS: Ocular-cicatricial pemphigoid is an autoimmune disease that, untreated, progresses to conjunctival scarring and blindness; systemic immunosuppression is required to control it. Long-term systemic treatment and more than one drug are frequently necessary to avoid recurrences, exposing elderly patients to a higher risk of drug toxicity. The most frequently encountered treatment-related side effects were anemia, leukopenia, liver toxicity, and hypertension.
PMID: 11772589 [PubMed - indexed for MEDLINE]
Saturday, February 3, 2007
The effect of treatment and its related side effects in patients with severe ocular cicatricial pemphigoid.
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Hi there! great stuff here, I'm glad that I drop by your page and found this very interesting. Thanks for posting. Hoping to read something like this in the future! Keep it up!
Ocular cicatricial pemphigoid is an auto immune disease affecting the conjunctiva. You will remember, from the first Patient Information entry on this Web Site, that auto immune diseases result from dysregulation of the patient's immune system, with the white blood cells becoming ""confused"" and beginning to attack not only germs but also part of the patient's own body. In rheumatoid arthritis, for example, the white blood cells become confused and begin to attack tissue in the patient's joints. In pemphigoid, the white blood cells attack skin and mucous membrane, particularly mucous membranes of the mouth, eyes, nose, throat, vagina, and rectum. Patients may or may not have more than one site affected by the disease, but 70% of the patients with cicatricial pemphigoid have eye involvement. And because this is a systemic autoimmune disease, it cannot be successfully treated, long-term, with simple topical (drops) therapy. It must be treated systemically, and typically with strategies that ""cool down"" the immune system, i.e., suppress the immune system sufficiently to stop the autoimmune process. Suppressing the immune system sufficiently to stop the autoimmune process for a short period (one to five years) is usually sufficient to enduce permanent remission of the disease. If this is not done, then continued inflammation, continued scarring, and eventual blindness in both eyes usually occurs.
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