抗SRP抗体陽性の重症多発性筋炎に免疫グロブリン大量療法(IVIG)が奏効した一例

• Published in: 臨床リウマチ Vol. 26; no. 1; pp. 63 - 68
• Main Authors: 田崎, 知江美, 木下, 浩二, 嶋津, 秀紀, 井上, 明日圭, 廣岡, 靖章, 朝戸, 佳世, 岸本, 和也, 樋野, 尚一, 志賀, 俊彦, 松村, 到, 船内, 正憲, 矢野, 智洋, 野﨑, 祐史, 李, 進海, 湯本, 妙子
• Format: Journal Article
• Language: Japanese
• Published: 一般社団法人 日本臨床リウマチ学会 2014; The Japanese Society for Clinical Rheumatology and Related Research
• Subjects: anti-SRP antibody; intravenous immunoglobulin therapy; polymyositis
• ISSN: 0914-8760; 2189-0595
• DOI: 10.14961/cra.26.63

    The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation, leading to progressive weakness of the proximal muscles. Myositis-specific or associated autoantibodies are often found in the serum of polymyositis (PM) and dermatomyositis patients. Anti-SRP (signal recognition particle) antibody is thought to be associated with severe forms of the disease, particularly those with heart and lung involvement and resistant to adrenocorticosteroids. We present a66-year-old female polymyositis patient with serious muscle weakness and high CPK level (21550IU/L). Despite initial therapy with high-dose methylprednisolone (1g/day x3days,i.v.) followed by prednisolone (1mg/kg/day,p.o.) plus cyclosporine A (150mg/day), muscle weakness was not improved and CPK levels were not reduced to less than3000IU/L. After treatment with intravenous immunoglobulin (IVIG), muscle strength was gradually improved and CPK levels were reduced to less than700IU/L. To our knowledge, there were few reports that PM positive for serum anti-SRP antibody treated with IVIG. IVIG might be a therapeutic agent of choice for steroid-resistant cases of serum anti-SRP antibody-positive PM.