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目的:报告1例54岁男性意义未明的单克隆丙种球蛋白病(MGUS)伴炎性肌病的患者。方法:回顾分析我院收治的1例MGUS伴炎性肌病患者的临床表现、肌肉病理改变和治疗,并结合复习国外文献报道的9例病例。结果:患者于52岁时出现不对称性双上肢远端无力,并逐渐加重。血清免疫电泳检查示IgM单株峰,且单克隆球蛋白(M-蛋白)浓度低于30 g·L-1,骨髓中浆细胞<10%,尿蛋白(-),肾功能正常,无贫血、高钙血症及溶骨性改变。肌活检提示炎性肌病可能。予丙种球蛋白、环磷酰胺、双膜法血浆置换治疗后症状短期有所改善,但总体仍不断进展。结论:MGUS可合并炎性肌病,可能与M-蛋白介导的自身免疫相关。
PURPOSE: To report a case of a 54-year-old male with unidentified monoclonal gammopathy (MGUS) with inflammatory myopathy. Methods: A retrospective analysis of 1 case of MGUS with inflammatory myopathy in patients with clinical manifestations, muscle pathology and treatment, combined with the review of 9 cases reported in foreign literature. Results: The patient developed asymmetry at age 52 with both upper limb weakness and worsening. Serum immunoelectrophoresis showed single peak of IgM, and the concentration of monoclonal globulin (M-protein) was lower than 30 g · L-1, plasma cells in bone marrow was less than 10%, urine protein (-), normal renal function, , Hypercalcemia and osteolytic changes. Muscle biopsy may indicate inflammatory myopathy. To gamma globulin, cyclophosphamide, double membrane plasma exchange therapy after symptoms improved in the short term, but the overall continued progress. Conclusion: MGUS may be associated with inflammatory myopathy and may be related to M-protein-mediated autoimmunity.