Clinical manifestations of Behcet's disease in Iran. Analysis of 2068 cases.


Abstract: The annual incidence of Behcet's Disease (BD) is as high as 300 patients per year. The mean age at the onset of the first symptom is 26.7 years (SD = 9.7). The sex distribution is 54% male and 46% female. The initial manifestation was mainly oral aphthosis (73%), uveitis (13%), genital ulcerations ­(11%), arthritis (9%), and skin manifestations (9%). Clinical manifestations were as fallow: mucous mem­brane manifestations were seen in 95.5% of patients (oral aphthosis in 95%, genital ulcerations in 63%), skin manifes­tations in 78% (folliculitis in 71%, erythema nodasam in 23%, other lesions in 1%). Ophthalmologic manifestations are the major cause of morbidity in BD, it was seen in 65% of patients (anterior uveitis in 51.5%, posterior uveitis in 51%, retinal vasculitis in 34%). Joint manifestations, usually transient, were seen in 48% of patients [arthralgia in 27%, monoarthritis (mainly large joints) in 11%, oligoarthritis in 22%, ankylosing spondylitis in 1.5% (the prevalence of SPA in the general population is 0.11%)]. Gastrointestinal mani­festations were seen in 10% of patients (gastroduodenitis in 42%, peptic ulcers in 2%, diarrhea in 2.3%, rectorrhagia in 0.9%, abdominal pain mimicking acute abdomen in 2.3%). Neurologic manifestations were seen in 12% of patients [central manifestations in 3%, peripheral manifestations in 0.2%, and headache in 10% (mainly of tension headache type)]. Orchiepididymitis was seen in 5.9% of patients (22% of male patients). Phlebitis was seen in 9.5% of patients. Ar­terial thrombosis was seen only in 1 case. Large vein throm­bosis was seen in 21 cases (1%). Pulmonary manifestation were seen in 17 cases (0.8%): vasculitis in 8, fibrosis in 2. pleuritis in 5, and infection in 5 cases. Cardiac manifestations were seen in 13 cases (0.6%), with angina pectoris in 2. myo­cardial infarction in 3, pericarditis in 6, and heart murmur in 3 cases. Aneurysm was detected only in 3 cases. Renal mani­festations were not rare (proteinuria in 3.6%, hematuria in 6%, and casts in 0.4% of patients). These manifestations were transitory. In 8 cases they were chronically present. The kidney biopsy showed a WHO type III glomerulonephritis in 4 patients and a type IV in 4 other patients. In 1 of the type III patients the increase of proteinuria led to a second biopsy which showed a secondary amyloidosis., not present on the first biopsy.