Behçet's disease in Portugal.

During the last decades 156 patients with Behçet's disease were diagnosed and studied in Portugal. Until 1966 only two cases were referred in Portugal. In 1978 we reported 23 cases (18 males and 5 females) with multisystemic involvement, not different from that reported in other publications elsewhere. In 1987, 17 cases were reported from the Oporto region, diagnosed during a period of 5 years. In the region of Coimbra 15 cases were diagnosed (6 males and 9 females). In the Lisbon area from 1969 until 1990, 123 cases were studied altogether, 76 during the last decade (35 males and 41 females). All the cases studied had oral ulcerations and a high incidence of ocular involvement (clinical and sub-clinical, 87%). HLA-B5 was detected in 50% of the patients. At least four cases were fatal, three died with neuro-Behçet. Steroids, colchicine and thalidomide were the main form of treatment, for the patients in the Lisbon area, but in some cases chlorambucil, cyclophosphamide, azathioprine, cyclosporine-A, fibrinolytic therapy, plasmapheresis and isovolemic hemodilution were also used.