免疫介导的坏死性肌病 (IMNM) 是自身免疫性肌炎，临床特征为近端肌无力和肌酸激酶 (CK) 升高。它们可能与自身抗体（抗 HMGCR、抗 SRP）有关、由他汀类药物使用引发（例如抗 HMGCR 肌病）、与癌症相关或可能是特发性的。需要免疫疗法来提高强度并降低 CK 水平，但目前美国食品和药物管理局尚未批准用于治疗 IMNM 的疗法。 IMNM 的最佳治疗策略目前尚不清楚，并且在这种情况的管理中存在广泛的实践差异。然而，观察性研究和专家意见表明，某些疗法可能对 IMNM 的不同血清学亚型更有效。 HMGCR IMNM 通常对静脉注射免疫球蛋白 (IVIG) 反应良好，即使作为单一疗法也是如此。信号识别肽和血清阴性 IMNM 通常需要联合免疫治疗，最常见的是口服免疫抑制剂、皮质类固醇和 IVIG 或利妥昔单抗。患者通常会持续多年接受免疫治疗，并且在逐渐减少免疫治疗期间复发很常见。需要进一步的研究来指导这些患者的最佳治疗。© 2024 美国神经肌肉协会

The immune-mediated necrotizing myopathies (IMNM) are autoimmune myositides clinically characterized by proximal predominant weakness and elevated creatine kinase (CK). They may be associated with autoantibodies (anti-HMGCR, anti-SRP), triggered by statin use (e.g., anti-HMGCR myopathy), associated with cancer, or may be idiopathic. Immunotherapy is required to improve strength and decrease the CK level, but no therapies are currently approved by the U.S. Food and Drug Administration for the treatment of IMNM. The optimal treatment strategy for IMNM is currently unknown and wide practice variation exists in the management of this condition. However, observational studies and expert opinion suggest that certain therapies may be more effective for the different serological subtypes of IMNM. HMGCR IMNM often responds favorably to intravenous immunoglobulin (IVIG) even as monotherapy. Signal recognition peptide and seronegative IMNM typically require combination immunotherapy, most often consisting of an oral immunosuppressant, corticosteroids, and IVIG or rituximab. Patients often remain on immunotherapy for years and relapse is common during tapering of immunotherapy. Further studies are needed to guide the optimal management of these patients.© 2024 by the American Association of Neuromuscular & Electrodiagnostic Medicine, Inc. All rights reserved.