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成人囊性纤维化中的发作性关节病

Episodic arthropathy in adult cystic fibrosis.

作者信息

Bourke S, Rooney M, Fitzgerald M, Bresnihan B

机构信息

Department of Respiratory Medicine, St Vincent's Hospital, Dublin, Ireland.

出版信息

Q J Med. 1987 Aug;64(244):651-9.

PMID:3444871
Abstract

Five (8.5 per cent) of 59 adult patients with cystic fibrosis described a characteristic arthropathy which was episodic, self-limiting and polyarticular, affecting large and small peripheral joints without evidence of progression to joint damage. The five patients with arthropathy did not differ from the remaining 54 with respect to manifestations of cystic fibrosis. Synovial fluid obtained from two patients during acute episodes was clear, viscous and contained no cells. Biopsies of synovial membrane from the same patients demonstrated prominent congested blood vessels with some synovial oedema, but no evidence of inflammation. Immunofluorescent staining of synovial membrane demonstrated characteristic intimal deposition of immunoglobulin in the vessels. In one of the two patients acute arthropathy was accompanied by transient cutaneous vasculitis, circulating immune complexes and lowered serum complement levels, which suggests that the episodic arthropathy in at least some patients with cystic fibrosis is mediated by an immune mechanism.

摘要

59例成年囊性纤维化患者中有5例(8.5%)描述了一种特征性关节病,该病呈发作性、自限性且累及多个关节,影响外周大小关节,无进展至关节损伤的证据。这5例患有关节病的患者与其余54例患者在囊性纤维化的表现方面并无差异。在急性发作期从2例患者获取的滑液清澈、黏稠,无细胞。同一患者的滑膜活检显示血管明显充血,有一些滑膜水肿,但无炎症迹象。滑膜的免疫荧光染色显示血管内膜有特征性免疫球蛋白沉积。在这2例患者中的1例,急性关节病伴有短暂性皮肤血管炎、循环免疫复合物及血清补体水平降低,这表明至少部分囊性纤维化患者的发作性关节病是由免疫机制介导的。

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