Enfermedad de Behçet en Chile: Análisis clínico de 44 casos / Retrospective review of 44: Chilean patients with Behçet disease

Background: Behget's disease (BD) is a rare multisystemic inflammatory disease that is potentially disabling and may cause death. Aim: To describe the characteristics of BD patients from two Chilean centers. Patients and method: Retrospective review of the clinical records of patients with BD attended in two rheumatology services between 1985 and 2007. The "Behget's Disease Research Committee of Japan" (BDCJ) and the "International Study Group for Behget's Disease" (ISG) diagnostic criteria were applied. Results: We found 44 cases (25 males), diagnosed as BD. The mean age at the onset of symptoms was 26± 12 years. According to BDCJ criteria, 13 patients had complete BD, 24 had incomplete BD and 7 had a suspected BD. Thirty two patients fulfilled the ISG criteria. Forty two patients (95 percent) had oral ulcers, 33 (75 percent) had genital ulcers and 29 (66 percent) had ophthalmological involvement. Eleven and three patients had symptoms of central and peripheral nervous system involvement, respectively. No gender differences were detected. Conclusions: The clinical characteristics of these patients were similar to those described abroad, except for a higher frequency of peripheral nervous system involvement and a lower rate of arthritis.

Eficacia de la azatioprina en la enfermedad ocular inflamatoria no infecciosa resistente a tratamiento esteroidal sistémico / Role ofAzathioprine in steroid resistant non infectious ocular inflammatory diseases

Background: Topical and systemic steroids are the first line of treatment of non infectious inflammatory ocular disease. Immunosuppresants are reserved as a second line treatment. Aim: To evaluate the role ofAzathioprine (AZA) as a coadyuvant immunosuppressive treatment for non infectious ocular inflammatory diseases (OIDs) resistant to systemic steroid therapy in a retrospective, noncomparative interventional case series. Patients and methods: Patients using oral Prednisone due to an active or recurrent OID, without clinical response, and not receiving any other immunosuppressive treatment were studied. A standard protocol of oral Prednisone (0.5 mg/kg/ day) and oral AZA (2-3 mg/kg/day) during one year was used. Ocular and systemic monthly evaluations were done including relapse rate, steroid dosage, inflammatory score and visual acuity. Results: Thirty patients (10 male) aged 18-75 years (mean 44 years) were studied. Three had bilateral anterior uveitis, one had pars planitis, four had diffuse uveitis, eight Vogt-Koyanahi-Harada syndrome, three Behget's disease, three necrotizing scleritis and eight had retinochoroidopathy A complete initial response was observed in 26 patients (87 percent). The time of response was between 1 to 6 months (mean 2.65 months). Seventeen percent of these had a relapse 6 to 12 months after AZA was started. In 61 percent, visual acuity improved. The ocular inflammatory score decreased in 86.5 percent. Eleven patients had mild controlled side effects that did not require discontinuation of AZA. Conclusions: Combined systemic steroid and oral AZA therapy is safe and effective in controlling steroid resistant non infectious inflammatory ocular diseases.