A reappraisal of amyloidosis in Behçet's syndrome

• Published in: Rheumatology (Oxford, England) Vol. 40; no. 2; pp. 212 - 215
• Main Authors: MELIKOGLU, M, ALTIPARMAK, M. R, FRESKO, I, TUNC, R, YURDAKUL, S, HAMURYUDAN, V, YAZICI, H
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.02.2001; Oxford Publishing Limited (England)
• Subjects: Adult; Amyloidosis - epidemiology; Amyloidosis - etiology; Behcet Syndrome - complications; Biological and medical sciences; Female; Humans; Medical sciences; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Glomerulonephritis; Skin disease; Prognosis; Cardiovascular disease; Enzymopathy; Epidemiology; Genital diseases; Vascular disease; Association; Systemic disease; Public health; Kidney disease; Human; Urinary system disease; Stomatology; Metabolic diseases; Nephrotic syndrome; Behçet syndrome; Protein; Symptomatology; Eye disease; Risk factor; Frequency; Amyloidosis; Proteinuria
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/40.2.212

To evaluate the clinical features and outcome of patients with Behçet's syndrome (BS) and amyloidosis and to assess the associated risk factors. A chart review was done to determine the frequency of amyloidosis in BS among 4000 patients. Data from 14 BS patients with amyloidosis were compared with data obtained from 718 patients with BS without amyloidosis. Multiple stepwise logistic regression analysis was used to assess the risk factors. All patients with amyloidosis presented with the nephrotic syndrome or significant proteinuria. The mean time to the onset of amyloidosis was 8.1 yr (range 3-15 yr). The mean duration of follow-up after amyloidosis was 3.4 yr (range 1-11 yr). Seven out of 14 patients were alive at the time of the evaluation. Peripheral and pulmonary arterial involvement and arthritis were associated with amyloidosis (P<0.05). Amyloidosis in BS is rare and has a 50% mortality rate at 3.4 yr (range 1-11 yr). Peripheral and pulmonary arterial involvement and arthritis seem to be the strongest predictors of amyloidosis in BS.