キシ　タカユキ   KISHI Takayuki   岸　崇之    所属   医学部 医学科（東京女子医科大学病院）    職種   講師
論文種別	原著
言語種別	英語
査読の有無	査読あり
表題	Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.
掲載誌名	正式名：Rheumatology (Oxford, England) 略　 称：Rheumatology (Oxford) ISSNコード：14620332/14620324
掲載区分	国外
巻・号・頁	pp.Online ahead of print.
著者・共著者	Sherman Matthew A, Graf Rose, Sabbagh Sara E, Galindo-Feria Angeles S, Pinal-Fernandez Iago, Pak Katherine, Kishi Takayuki, Flegel Willy A, Targoff Ira N, Miller Frederick W, Lundberg Ingrid E, Rider Lisa G, Mammen Andrew L,
発行年月	2022/08
概要	OBJECTIVES:Four-and-a-half LIM domains 1 (FHL1) is a muscle-specific protein. Autoantibodies against FHL1 were recently discovered in adults with idiopathic inflammatory myopathies (IIM) and were found to be associated with clinical features and outcomes indicative of increased disease severity. Anti-FHL1 autoantibodies have not been described in children. Here, the prevalence and clinical features associated with anti-FHL1 autoantibodies were examined in a large North American cohort of juvenile patients with IIM.METHODS:Sera from 338 juvenile IIM patients and 91 juvenile healthy controls were screened for anti-FHL1 autoantibodies by ELISA. Clinical characteristics and HLA alleles of those with and without anti-FHL1 autoantibodies were compared among those with juvenile IIM.RESULTS:Anti-FHL1 autoantibodies were present in 10.9% of juvenile IIM patients and 1.1% of controls. The frequency of anti-FHL1 autoantibodies among clinical and serologic subgroups did not differ. A higher percentage of Asian patients had anti-FHL1 autoantibodies (11% vs 0.7%, p= 0.002). Myositis associated autoantibodies (MAAs) (OR 2.09; CI 1.03, 4.32), anti-Ro52 autoantibodies specifically (OR 4.17; CI 1.83, 9.37), and V-sign rash (OR 2.59; CI 1.22, 5.40) were associated with anti-FHL1 autoantibodies. There were no differences in other features or markers of disease severity. No HLA associations with anti-FHL1 autoantibodies in Caucasian myositis patients were identified.CONCLUSION:Anti-FHL1 autoantibodies are present in ∼11% of juvenile IIM patients and commonly co-occur with MAAs, including anti-Ro52 autoantibodies. In contrast to adult IIM, anti-FHL1 autoantibodies in juvenile myositis are associated with V-sign rash but not with other distinctive clinical features or worse outcomes.
DOI	10.1093/rheumatology/keac428
PMID	35961028