摘要目的 探讨抗信号识别颗粒抗体阳性坏死性肌病患者的临床表现、肌肉病理特点和治疗. 方法 济南军区总医院神经内科自2011年3月至2012年7月收治5例抗信号识别颗粒抗体阳性坏死性肌病患者,回顾性分析这5例患者的临床表现、肌肉病理特点和治疗方法. 结果 5例患者呈亚急性起病、四肢近端肌无力,血肌酸激酶均显著升高,血清中抗信号识别颗粒抗体阳性,肌电图均提示肌源性损害.肌肉标本病理表现为变性、坏死伴再生,可见萎缩及肥大肌纤维,伴结缔组织增生,未见明显炎性细胞浸润;免疫组化染色可见CD4阳性T淋巴细胞及CD68阳性的巨噬细胞浸润坏死肌纤维,未见CD8阳性T淋巴细胞及CD20阳性B淋巴细胞;肌聚糖蛋白、抗肌萎缩蛋白及dysferlin染色均呈连续强阳性表达.患者对糖皮质激素治疗反应均差,联合使用免疫抑制剂比单用激素治疗效果好. 结论 抗信号识别颗粒抗体阳性坏死性肌病的临床特点为对称性肢体近端肌无力,伴血清肌酸激酶明显升高,病理特点为肌纤维坏死伴再生,无或较少炎性细胞浸润,对糖皮质激素或免疫抑制剂治疗效果欠佳.

abstractsObjective To investigate the clinical and pathological features of necrotizing myopathy with anti-signal recognition particle antibodies and its treatment.Methods Five patients with necrotizing myopathy with anti-signal recognition particle antibodies,admitted to our hospital from March 2011 to July 2012,were chosen in our study; their clinical and pathological manifestations and treatment methods were retrospectively analyzed.Results Five patients showed clinical manifestations of subacute onset,proximal limb muscle weakness.Serum creatine kinase level was significantly elevated.Immunoblotting confirmed positive signal recognition particle antibodies.EMG prompted myogenic damage.Pathology manifested as muscle degeneration,necrosis with regeneration,visible atrophy and hypertrophy of muscle fibers,and connective tissue hyperplasia,and no significant inflammatory cell infiltration was noted.Immunohistochemistry staining showed necrotizing muscle fibers infiltrated with CD4-positive T lymphocytes and CD68-positive macrophages,no CD8-positive T lymphocytes and CD20-positive B lymphocytes.Sarcoglycans staining,dystrophine staining and dysferlin staining showed continuous strong positive expression.Follow-up found patients were poor response to glucocorticoid,and a combination therapy of immunosuppressive agents was better than hormone alone.Conclusion The prominent manifestations of necrotizing myopathy with anti-signal recognition particle antibodies are symmetric proximal muscle weakness,with highly elevated levels of serum creatine kinase; fiber necrosis and regeneration are the main myopathlogical features,without obvious inflammatory cells infiltrates; this disease is poorly responsive to corticosteroids and immunosuppression.