Management of immune-mediated necrotizing myopathy

被引:0
作者
Suh, Joome [1 ]
Amato, Anthony A. [1 ]
机构
[1] Harvard Med Sch, Brigham & Womens Hosp, Dept Neurol, Boston, MA 02115 USA
关键词
HMGCR myopathy; immune-mediated necrotizing myopathy; necrotizing autoimmune myopathy; SRP myopathy; statin myopathy; CLINICAL-FEATURES; ANTIBODIES; AUTOANTIBODIES; MYOSITIS; OUTCOMES; DISEASE; COHORT;
D O I
10.1002/mus.28114
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:166 / 172
页数:7
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