Suemitsu T, Saga T, Inui A, Ariizumi M, Shogi E, Sato H
Department of Pediatrics, Dokkyo University, School of Medicine, Koshigaya Hospital, Saitama, Japan.
Nihon Jinzo Gakkai Shi. 1993 Feb;35(2):189-94.
A case of Behçet's disease with IgA nephropathy was reported in a 17 year old man. He presented with microscopic hematuria which was pointed out by urinary mass screening for school children at age of 14. As he was subsequently evolved proteinuria, a renal biopsy was performed at age of 16. The specimen showed diffuse proliferation of mesangial cells. Immunofluorescent studies showed granular deposition of IgA. At age of 17, he showed typical symptoms of Behçet's disease, namely aphthous stomatitis, perianal ulcers, erythema nodosum-like lesions, uveitis and genital aphtha. Furthermore he showed an increasing of proteinuria and an impairment of his renal function. Serum IgA level and immune complexes during the exacerbation stage were elevated. The uveitis markedly improved with ciclosporin A therapy. Reduction of proteinuria and improvement of renal function were simultaneously observed. These findings may suggest that IgA nephropathy and Behçet's a disease have common immunological pathogenesis, including circulating immune complexes.
一名17岁男性被报道患有白塞病合并IgA肾病。他在14岁时接受学校儿童尿液大规模筛查时被发现有镜下血尿。随后他出现蛋白尿，16岁时进行了肾活检。标本显示系膜细胞弥漫性增生。免疫荧光研究显示IgA呈颗粒状沉积。17岁时，他出现了白塞病的典型症状，即口腔溃疡、肛周溃疡、结节性红斑样病变、葡萄膜炎和生殖器溃疡。此外，他的蛋白尿增加且肾功能受损。病情加重期血清IgA水平和免疫复合物升高。葡萄膜炎经环孢素A治疗后明显改善。同时观察到蛋白尿减少和肾功能改善。这些发现可能提示IgA肾病和白塞病有共同的免疫发病机制，包括循环免疫复合物。
