自己抗体を介する炎症性筋疾患の臨床像・筋病理の解析と病態機序の解明

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免疫介在性壊死性ミオパチー(immune-mediated necrotizing myopathy, IMNM)に関連し抗signal recognition particle(SRP)抗体と抗3-hydroxy-3-methylglutary-coenzyme A reductase(HMGCR) 抗体の意義を明らかにした。IMNM患者において抗SRP抗体が39％, 抗HMGCR抗体が25%で検出され, 抗SRP抗体は抗HMGCR抗体と比べ, 重症例が多かった。HLA-DRB1遺伝子解析からDRB1*08:03と抗SRP抗体, DRB1*11:01と抗HMGCR抗体の関連を証明した。 Immune-mediated necrotizing myopathy (IMNM) is a pathological entity characterized by necrosis in the absence of prominent endomysial lymphocytic infiltration. Autoantibodies against signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with IMNM. The frequencies of anti-SRP and anti-HMGCR antibodies in patients with IMNM were 39% and 25%, respectively. Severe limb muscle weakness, neck weakness, dysphagia, respiratory insufficiency, and muscle atrophy were more frequently observed in patients with anti-SRP antibodies than with anti-HMGCR antibodies. HLA-DRB1 genotyping revealed the association of anti-SRP antibodies with DRB1*08:03 and that of anti-HMGCR antibodies with DRB1*11:01.
研究種目 : 基盤研究(C)(一般) 研究期間 : 2014～2016 課題番号 : 26461298 研究分野 : 神経内科学