title: [Systemic treatment of ocular cicatricial pemphigoid].
abstract: Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppressive drugs. Four patients combined two immunosuppressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.
authors.affiliation: Servicio de Alergia e Inmunología, Hospital Británico de Buenos Aires, Argentina. mceciliaj@yahoo.com.ar
authors.forename: María Cecilia
authors.initials: MC
authors.lastname: Juri
authors.forename: Diego S
authors.initials: DS
authors.lastname: Fernández Romero
authors.forename: Martín H
authors.initials: MH
authors.lastname: Devoto
authors.forename: Daniel
authors.initials: D
authors.lastname: Georgiett
authors.forename: Jorge O
authors.initials: JO
authors.lastname: Zárate
authors.forename: Alejandro
authors.initials: A
authors.lastname: Malbrán
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000188:drug therapy*; D001379:Azathioprine / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D057210:Delayed Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000473:pathology; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003622:Dapsone; D001379:Azathioprine; D008727:Methotrexate