BEHCETS-DISEASE IN BRAZIL - A REVIEW OF 49 CASES WITH EMPHASIS ON OPHTHALMIC MANIFESTATIONS

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Data
1991-01-01
Autores
Barra, C.
Belfort, Rubens Junior [UNIFESP]
Abreu, Mariza Toledo de [UNIFESP]
Kim, M. K.
Martins, M. C.
Petrilli, Ana Maria Noriega [UNIFESP]
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At the Uveitis Clinic of the Department of Ophthalmology of the Escola Paulista de Medicina, Sao Paulo, Brazil, Behcet's disease was diagnosed in 49 patients, representing 2.0% of the total uveitis cases attended during the 16-year period from February 1974 to June 1990. Of these, 71% were men. The age of onset of the disease was between 9 and 61 years, with a mean of 29.6 years. The ethnic distribution was the following. 76% Caucasian, 14% darkly pigmented, 8% Mongoloid and 2% Negroid. HLA-B5 was found in 45.5% of the 11 Caucasian patients typed. In 34.5% of the cases, the ocular attack was the initial manifestation, alone or associated with other symptoms. Oral aphthae were recorded in 98% of the patients, genital ulcers in 55.1%, and skin lesions in 51%. Joint involvement was present in 44.9%, neurologic symptoms were evidenced in 3 patients, and 2 patients had major vascular involvement. The mean interval between the first manifestation of the disease and the onset of ocular involvement was 3.1 years, with a range of 4 months to 14 years. The interval between affections of both eyes ranged from 0 to 2 years; in 38.4% of the cases it occured within one month. Anterior and posterior segment involvement was seen in 85.7% of the patients. Hypopyon was observed in 34.7% of the cases. Seven patients did not present iridocyclitis at any time in the course of their disease. We did not see any cases with only anterior segment involvement. Retinal vasculitis was present in all cases; macular involvement was found in 26.5% of the cases, papillitis in 16.3%, and venous occlusion in 12.2%. Ocular complications included: cataract in 40 of the 96 eyes of the 49 patients, optic atrophy in 18, macular degeneration in 15, macular hole in 5, and phthisis bulbi in 2 eyes. The most frequent therapeutic modality used in these patients was prednisone with a cytotoxic (alkylating) agent, sometimes also in combination with colchicine (75.6%). Ten (21.3%) patients showed permanent visual impairment of 20/400 or worse in both eyes, at an average of 3.5 years after the initiation of the ocular symptoms. However, a visual acuity of 20/40 or better was retained in 38 of the 96 eyes involved (39.6%) after a follow-up period of 6 months to 8.5 years, with a mean of 3 years.
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Japanese Journal Of Ophthalmology. Hongo Tokyo: Japan J Ophthalmol, v. 35, n. 3, p. 339-346, 1991.