Objective: To assess the frequency and clinical manifestations of Behçet's disease (BD) during the past 10 years in a defined area of Northwestern Spain.
Methods: The charts of all the patients diagnosed as having BD at the Hospital Xeral-Calde from 1988 through 1997 were reviewed. All the patients were residents of Lugo. Patients were followed from the time of diagnosis until either the patient's death or July 1, 1998. By that time patients were classified as having complete BD if they fulfilled the International Study Group Criteria (ISG) for BD. Patients with recurrent oral ulcerations plus one of the remaining 4 criteria of the ISG were classified as having an incomplete form of BD.
Results: Sixteen patients (9 men/7 women) were diagnosed as having complete BD and 7 (3 men) as having incomplete BD. The average annual incidence rate of complete BD in the Lugo region of Northwestern Spain was 0.66/100,000. Oral ulcerations with or without genital ulcerations were the most common initial manifestations. Neurological involvement was relatively frequent in men with complete BD. In contrast, a positive pathergy test was less common. Two patients with neurological involvement and 3 with uveitis had severe sequelae. However, there was a single death, apparently not related to BD.
Conclusion: In Northwestern Spain, BD is more common than was expected and is a nonfatal disease. However, central nervous system involvement and severe ocular complications overshadow the good prognosis observed in the majority of patients.