土耳其及全球范围内与淀粉样变性相关的白塞病。

Dilşen N, Koniçe M, Aral O, Erbengi T, Uysal V, Koçak N, Ozdogan E

Division of Rheumatology, Istanbul Faculty of Medicine, University of Istanbul, Capa.

Ann Rheum Dis. 1988 Feb;47(2):157-63. doi: 10.1136/ard.47.2.157.

The association of amyloidosis with Behçet's disease has infrequently been reported in published works. Twenty four such cases have been observed in the world, of which 12 are from Turkey, including eight of ours. In all our eight cases renal biopsy showed amyloidosis of type AA. Behçet's disease of male preponderance, long duration, complete type, multiple organ involvement, and positive skin pathergy test were the main characteristics of all 24 cases of Behçet's disease with amyloidosis. We conclude that amyloidosis associated with Behçet's disease is a secondary AA amyloidosis occurring as an intrinsic manifestation of Behçet's disease.

淀粉样变性与白塞病的关联在已发表的文献中鲜有报道。全球已观察到24例此类病例，其中12例来自土耳其，包括我们的8例。我们所有8例病例的肾活检均显示为AA型淀粉样变性。男性占优势、病程长、完全型、多器官受累以及皮肤针刺试验阳性是所有24例合并淀粉样变性的白塞病病例的主要特征。我们得出结论，与白塞病相关的淀粉样变性是作为白塞病固有表现而发生的继发性AA型淀粉样变性。