神経治療学
Online ISSN : 2189-7824
Print ISSN : 0916-8443
ISSN-L : 2189-7824
教育講演
炎症性筋疾患の治療update
清水 潤
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ジャーナル フリー

2020 年 37 巻 4 号 p. 537-542

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The idiopathic inflammatory myopathies (IIMs, also known as myositis) are a heterogeneous group of autoimmune disorders involving skeletal muscle. IIMs are mainly characterized with associated clinical complications, pathological findings, and myositis autoantibodies. It has been known that about 70 percent of patients have myositis autoantibodies ; myositis–specific autoantibodies (MSAs) or myositis–associated autoantibodies (MAAs). Because a number of MSAs/MAAs correlate with specific clinical features of patients with IIMs, and only one of MSAs is detected in individual patients, it is presumed that autoantibodies or their target molecules correlate with underlying pathomechanisms of IIMs.

For the treatment of IIM, high–dose oral corticosteroid therapy is used for the first–line therapy mainly based on experience. As second–line therapies, immunosuppressants are used as steroid–sparing agents or when the disease is refractory. Intravenous immunoglobulin therapy is considered in the patients who failed to respond to first–line therapy. Therefore, at present, most of the immunotherapies applied to IIMs patients are nonspecific in spite of heterogeneity of underlying pathological mechanisms. Currently, numerous synthetic and biological immunosuppressive agents are available to treat rheumatic diseases including IIMs. To develop more specific therapies targeting underlying pathogenic pathways, further studies on better classification of IIMs and further therapeutic studies on subsets of classified IIM patients should be necessary.

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