Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 +/- 11 S.D. yr (range 17-59 yr). Their mean disease duration was 5 +/- 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 +/- 11 S.D. months (range 1-36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 +/- 24 S.D. months (range 1-78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment.

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Objective-To make a retrospective and

SUMMARY Radiological sacroiliitis in Behqet's syndrome (BS) has been a subject of controversy.We have examined pelvic radiographs of 38 patients with BS and 28 age and sex matched controls which we reported previously, and also 17 with ankylosing spondylitis (AS), 27 with non-renal familial Mediterranean fever (FMF), and 33 with primary osteoarthrosis (OA). Initially, five observers assessed radiographs on two different occasions according to the New York criteria for sacroiliitis in a blind protocol. Later, three of them examined the various possible abnormalities of the sacroiliac (SI) joints after training sessions. Although the inter-and intraobserver variation was quite high, all observers found the expected changes in patients with AS. The abnormalities detected in the other diseases were either mild, inconsistent, or both. Erosions were confined to patients with AS, and osteophytes and glenoid sulci to patients with OA. We conclude that high observer variation in interpreting a film of the anteroposterior (AP) view of the pelvis for sacroiliitis may be a major cause of reported 'sacroiliitis' in BS and FMF.

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