A reappraisal of amyloidosis in Behçet's syndrome.
OBJECTIVE
To evaluate the clinical features and outcome of patients with Behçet's syndrome (BS) and amyloidosis and to assess the associated risk factors.


METHODS
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.


RESULTS
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).


CONCLUSIONS
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.
Melikoğlu
M
M
Department of Rheumatology, Cerrahpaşa Medical School, University of Istanbul, Istanbul, Turkey.
Altiparmak
M R
MR
Fresko
I
I
Tunç
R
R
Yurdakul
S
S
Hamuryudan
V
V
Yazici
H
H
D000328:Adult; D000686:Amyloidosis / Q000453:epidemiology / Q000209:etiology*; D001528:Behcet Syndrome / Q000150:complications*; D005260:Female; D006801:Humans