抗信号识别颗粒抗体肌病5例

收集中南大学湘雅医院老年神经内科2015年12月至2018年6月诊断为抗信号识别颗粒抗体肌病的患者5例。 患者均亚急性或慢性起病，临床表现为逐渐进展的对称性肢体近端无力，血清肌酸肌酶明显增高，血清抗信号识别 颗粒抗体阳性，肌电图提示肌源性损害。肌肉活检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 织增生及少量炎性细胞浸润；免疫组织化学染色可见CD4及CD8阳性淋巴细胞、CD68阳性巨噬细胞浸润；未见CD20 阳性淋巴细胞及CD303阳性树突细胞浸润；其中2例患者少量毛细血管C5b-9表达阳性；全部患者的抗肌聚糖蛋白染 色、抗Dysferlin染色、抗dystrophin染色连续强阳性表达。患者均对糖皮质激素治疗有效，部分患者需联合免疫球蛋白 及免疫抑制剂治疗。

Myopathy associated with anti-signal recognition peptide antibodies: Five case reports

Five patients with myopathy associated with anti-signal recognition peptide antibodies, admitt ed to our hospital from December 2015 to June 2018, were chosen in our study, and their clinical and pathological manifestations and treatments were retrospectively analyzed. Five patients showed subacute or chronic onset and proximal limb muscle weakness. Serum creatine kinase level was signifi cantly elevated. Immunoblott ing assay confi rmed the positive anti-signal recognition particle antibody. EMG prompted myogenic damage. Pathological features included muscle degeneration,necrosis with regeneration, visible atrophy and hypertrophic of muscle fiber, connective tissue hyperplasia and a small amount of inflammatory cell infiltration. Immunohistochemical staining showed necrotizing muscle fiber infiltrated with CD4-positive and CD8-positive lymphocytes and CD68-positive macrophages, and no CD20-positive lymphocytes and CD303-positive dendritic cells were observed. Two patients had expressed a bit of c5b-9 positive capillary. Antisarcoglycans staining, anti-dysferlin staining and dystrophin staining showed continuous strong positive expression. Follow-up study found that all patients were response to glucocorticoid, and a combination therapy of immunoglobulin and immunosuppression were necessary for some patients.