Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.
Anti-FHL1 autoantibodies in juvenile myositis are associated with anti-Ro52 autoantibodies but not with severe disease features.
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青少年肌炎中的抗 FHL1 自身抗体与抗 Ro52 自身抗体相关，但与严重疾病特征无关。
DOI：
10.1093/rheumatology/keac428
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发表时间：
2023
期刊：
影响因子：
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通讯作者：
ChildhoodMyositi
中科院分区：
文献类型：
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作者：
Sherman,MatthewA;Graf,Rose;Sabbagh,SaraE;Galindo-Feria,AngelesS;Pinal-Fernandez,Iago;Pak,Katherine;Kishi,Takayuki;Flegel,WillyA;Targoff,IraN;Miller,FrederickW;Lundberg,IngridE;Rider,LisaG;Mammen,AndrewL;ChildhoodMyositi
目的四个半LIM结构域1（FHL 1）是一种肌肉特异性蛋白。最近在患有特发性炎性肌病（IIMs）的成人中发现了针对FHL 1的自身抗体，并且发现其与指示疾病严重程度增加的临床特征和结果相关。抗FHL 1自身抗体尚未在儿童中描述。在这里，与抗FHL 1自身抗体的患病率和临床特征进行了研究，在一个大型的北美队列的青少年患者IIM.MethodsSera从338青少年IIM患者和91青少年健康对照进行了筛选抗FHL 1自身抗体ELISA。临床特点和HLA等位基因的那些与青少年IIM. ResultsAnti-FHL 1自身抗体和无抗FHL 1自身抗体进行了比较，存在于10.9%的青少年IIM患者和1.1%的对照。抗FHL 1自身抗体的频率在临床和血清学亚组之间没有差异。亚裔患者中抗FHL 1自身抗体的比例较高（11%vs0.7%，P= 0.002）。肌炎相关自身抗体（MAA）[比值比（OR）2.09（CI 1.03，4.32）]、特异性抗Ro 52自身抗体[OR 4.17（CI 1.83，9.37）]和V征皮疹[OR 2.59（CI 1.22，5.40）]与抗FHL 1自身抗体相关。在疾病严重程度的其他特征或标志物方面没有差异。没有HLA协会与抗FHL 1自身抗体在高加索人肌炎patients. ConclusionAnti-FHL 1自身抗体存在于约11%的青少年IIM患者和常见的共同发生的MAA，包括抗Ro 52自身抗体。与成人IIM相比，青少年肌炎患者的抗FHL 1自身抗体与V征皮疹相关，但与其他独特的临床特征或更差的结局无关。
ObjectivesFour-and-a-half LIM domains 1 (FHL1) is a muscle-specific protein. Autoantibodies against FHL1 were recently discovered in adults with idiopathic inflammatory myopathies (IIMs) 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.MethodsSera 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.ResultsAnti-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%vs0.7%;P= 0.002). Myositis-associated autoantibodies (MAAs) [odds ratio (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.ConclusionAnti-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.