Department of Neurology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Muscle Nerve. 2024 Aug;70(2):166-172. doi: 10.1002/mus.28114. Epub 2024 May 27.
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.
免疫介导的坏死性肌病（IMNM）是一种自身免疫性肌炎，临床上以近端为主的无力和肌酸激酶（CK）升高为特征。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（如抗 HMGCR 肌病），与癌症有关，也可能是特发性的。免疫治疗是改善肌力和降低 CK 水平所必需的，但目前没有任何疗法被美国食品和药物管理局批准用于治疗 IMNM。IMNM 的最佳治疗策略目前尚不清楚，这种疾病的管理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 的不同血清亚型更有效。HMGCR IMNM 即使作为单一疗法，也常对静脉注射免疫球蛋白（IVIG）有良好反应。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、IVIG 或利妥昔单抗。患者通常需要长期接受免疫治疗，免疫治疗减量期间常复发。需要进一步的研究来指导这些患者的最佳治疗。
