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炎症性肌病作为冷球蛋白血症性血管炎的首发表现。

Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis.

作者信息

Rodríguez-Pérez Noelia, Rodríguez-Navedo Yerania, Font Yvonne M, Vilá Luis M

机构信息

Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico.

出版信息

BMJ Case Rep. 2013 Jun 3;2013:bcr2013010117. doi: 10.1136/bcr-2013-010117.

Abstract

Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy.

摘要

冷球蛋白血症性血管炎的特征是免疫球蛋白在低温下沉积。最常见的表现为皮肤受累、关节痛、雷诺现象、周围神经病变和肾脏疾病。肌病并不常见,仅有少数病例报道。在此,我们报告一名31岁女性,她出现了累及上下肢的进行性肌无力、吞咽困难、感觉异常和可触及的紫癜。诊断性检查显示肌酸激酶升高,肌电图和神经传导研究提示弥漫性肌病和感觉运动性轴索性神经病变,肌肉活检提示炎性肌病。冷球蛋白水平两次升高。她对环磷酰胺和大剂量皮质类固醇治疗反应良好。环磷酰胺持续使用1年,随后使用甲氨蝶呤。泼尼松逐渐减量,1年后停用。随访4年后她仍处于临床缓解状态。该病例提示,对于出现炎性肌病的患者,鉴别诊断时应考虑冷球蛋白血症。

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