抗HMGCR/SRP抗体阳性的特发性炎性肌病特点分析

目的:探讨抗HMGCR/SRP抗体阳性的特发性炎性肌病(IIM)的临床和病理特点。方法:收集HMGCR/SRP抗体阳性的IIM患者5例,对其危险因素、临床表现、实验室检查、肌电图、肌肉MRI、肌肉病理、肌炎自身抗体及药物治疗进行分析。结果:抗HMGCR/SRP抗体阳性的IIM患者临床变异较大,但大多有肌肉无力;血清肌酸激酶均较高;5例患者肌电图均表现为典型的肌源性损害。肌肉MRI主要表现为肌肉水肿。3例为典型坏死性肌病表现,1例镜下偶见肌细胞坏死,1例为多发性肌炎表现。HMGCR抗体阳性患者其中1例为正服用他汀药物,另一患者服用抗精神病药物;SRP抗体阳性患者1例为自身免疫性疾病患者,1例长期服用他汀,另1例病因未明确。激素治疗效果不一,2例加用丙种球蛋白治疗,1例加用免疫抑制剂治疗。结论:抗HMGCR/SRP抗体阳性的IIM临床表现各异,肌电图仅能定位肌肉损害,主要依靠肌肉活检,肌炎自身抗体检查更有助于诊断和具体分型,疗效各型不一。

Characteristics of Idiopathic Inflammatory Myopathy with Anti-HMGCR or Anti-SRP Antibodies

To explore clinical and pathological characteristics of idiopathic inflammatory myopathy with anti-HMGCR or anti-SRP antibodies. Methods: Total 5 patients positive for anti-HMGCR/anti-SRP antibody with idiopathic inflammatory myopathy were enrolled in this study. Risk factors, clinical manifestations, laboratory findings, electromyography, muscle MRI, muscle pathology, myositis autoantibody, and drug treatment information were collected and analyzed. Results: Idiopathic inflammatory myopathy patients that were anti-HMGCR/anti-SRP antibody-positive displayed a large variation in clinical symptoms with most patients showing muscle weakness. Serum creatine kinase level was elevated in all patients. Electromyographical studies showed typical myogenic impairments in all patients. Muscle MRI was mainly characterized by muscle edema. Pathological manifestations of muscle biopsy showed necrotizing myopathy in 3 patients that were anti-HMGCR/anti-SRP antibody-positive, occasionally seen muscle fiber necrosis in 1 patient, and polymyositis in 1 patient. Of the 2 patients that were anti-HMGCR antibody-positive, 1 was taking statin, and the other was taking antipsychotics. Of the 3 patients that were anti-SRP antibody-positive, 1 patient had autoimmune disease, 1 patient had been taking statin long-term, and the other patient had no clear cause of disease. Results of hormone therapy varied; 2 patients received gamma globulin therapy, and 1 patient received immunization inhibitor treatment. Conclusion: The clinical manifestations of idiopathic inflammatory myopathy with anti-HMGCR/anti-SRP antibody are different. EMG can only be used to assess muscle damage, and muscle biopsy and myositis autoantibodies antibody are more helpful for diagnosis and determination of myopathy type. The curative effect varies from type to type.