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韦格纳肉芽肿伴双侧坏死性巩膜炎、多关节炎及肾衰竭经免疫抑制治疗有效。

Wegener's granulomatosis with bilateral necrotizing scleritis, polyarthritis and renal failure efficiently treated with immunosuppressive therapy.

作者信息

Pavelka K, Dostal C, Rossmann P, Kraus H, Matousovic K, Vrabec J

出版信息

Clin Rheumatol. 1986 Jan;5(1):112-7. doi: 10.1007/BF02030979.

Abstract

A case report of a female patient with Wegener's granulomatosis is presented. After an initial involvement of the upper respiratory tract in the form of a sinusitis, there followed a severe necrotizing bilateral scleritis necessitating the enucleation of the left eye ball. Renal involvement developed as late as 24 months after the onset of the disease and led to renal failure within three months. Throughout the duration of her disease, the patient had joint symptoms in the form of episodes of migratory nondeforming polyarthritis. The administration of corticosteroids alone in daily doses up to 60 mg prednisone failed to control the progression of the disease, while immunosuppressive therapy with cyclophosphamide combined with methylprednisolone pulse therapy and haemodialysis resulted in a marked improvement of renal function and in the subsidence of the ocular and articular symptoms.

摘要

本文报告一例女性韦格纳肉芽肿病患者。患者最初以上呼吸道鼻窦炎形式发病,随后出现严重的双侧坏死性巩膜炎,左眼眼球不得不摘除。肾脏受累直到疾病发作24个月后才出现,并在三个月内导致肾衰竭。在整个病程中,患者有关节症状,表现为游走性非变形多关节炎发作。单独使用皮质类固醇,每日剂量高达60毫克泼尼松,未能控制疾病进展,而环磷酰胺免疫抑制治疗联合甲基泼尼松龙冲击治疗及血液透析,使肾功能显著改善,眼部和关节症状消退。

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