Myositis-specific and myositis-associated autoantibodies in a large Indian cohort of inflammatory myositis
Myositis-specific and myositis-associated autoantibodies in a large Indian cohort of inflammatory myositis
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DOI：
10.1016/j.semarthrit.2020.10.014
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发表时间：
2021-02-01
影响因子：
5
通讯作者：
Aggarwal, Rohit
中科院分区：
文献类型：
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作者：
Gupta, Latika;Naveen, R.;Aggarwal, Rohit
背景资料：特发性炎症性肌炎（IIM）是一种异质性疾病，具有与特异性肌炎特异性抗体（MSA）和肌炎相关抗体（MAA）相关的不同临床表型。成人和青少年IIM（2017年ACR/EULAR标准）于2017年至2020年在印度北方的一家三级中心的MyoCite队列中招募。从数据库档案中提取标准化的临床和实验室变量。通过Line免疫测定法评价血清样品中是否存在MSA/MAA，并通过免疫荧光测定法（IFA）评价抗核抗体（ANA）。结果：在250例IIM患者（214例成人，36例儿童）的年龄[40（30 - 49），13（7.5-16）岁]和疾病持续时间[ 7（3-17），6（2-17）个月]，主要包括皮肌炎（DM），其次是重叠肌炎（OM）的MSA和MAAs进行了测试。在148例（59.2%，60.7%成人和50% BIM）中发现了MSA/MAA，其中三分之二为MSA（95，64%）。2例（0.8%）有一个以上的MSA。在成人IIM中，最常见的MSA是抗Jo-1（10%），而在青少年IIM（JIM）中，抗MDA 5和抗NXP 2各4（11%）。ANA阳性率为76.0%（172/226），以斑点状最常见（37%）。ANA阴性者中近三分之二（54，61%）患有MSA/ MAA。近一半（18/54，54.6%）的MSA与胞浆型相关。抗氨酰-tRNA合成酶（ARS）与机械手（OR-7.06）、ILD（OR-4.4）和关节炎（OR-2.23）相关。抗Jo-1和非Jo-1抗合成酶综合征（ASS）的临床相关性没有差异。抗MDA-5与成人口腔溃疡（OR-8.3）、发热（OR-8.6）和体重减轻（OR-7.35）相关，与儿童关节炎（OR-11.5）和甲周皮疹（OR-9.6）相关。抗TIF-1 γ与光敏性（OR-10.44）和恶性肿瘤（OR-34）在成人，表皮过度生长（OR-11.2）在childhen.Conclusion：肌炎自身抗体被认为是在三分之二的ILMs和与不同的临床子集。Jo-1和非Jo-1 ASS表现出相似的特征。在成人中证实了抗TIF 1 γ与恶性肿瘤的相关性。MSA/MAA存在于ANA阴性的患者中的三分之二，MSA几乎总是相互排斥的。(C)2020爱思唯尔公司All rights reserved.
Background: The Idiopathic Inflammatory Myositis (IIM) are heterogenous with distinct clinical phenotypes associated with specific myositis specific antibodies (MSA) and myositis associated antibodies (MAA).Objectives: To evaluate the frequency, pattern and associations of MSA/MAA in a large Indian cohort of IIM.Methods: Adult and juvenile IIM (2017 ACR/EULAR criteria), were recruited in the MyoCite cohort between 2017and 2020 at a tertiary center in Northern India. Standardized clinical and laboratory variables were extracted from the database archive. Serum samples were evaluated for the presence of MSAs/MAAs by Line immunoassay and anti-nuclear antibodies (ANA) by Immunofluorescence assay (IFA). The prevalence and clinical associations of different MSA/MAAs were assessed.Results: MSA and MAAs were tested in 250 IIM patients (214 adults, 36 children) of age [40 (30 49), 13 (7.5-16) years] and disease duration [ 7 (3-17), 6 (2-17) months] comprising predominantly of Dermatomyositis (DM) followed by Overlap myositis (OM). MSAs/MAAs were found in 148 (59.2%, 60.7% adults and 50% BIM), of which two-thirds were MSA (95, 64% overall). Two cases (0.8%) had more than one MSA. In adult IIM, the most common MSA was anti-Jo-1 (10%), whereas it was anti-MDA5 and anti-NXP2 4 (11%) each in Juvenile IIM (JIIM). 76.0% (172/226) were ANA positive, with speckled pattern being the most common (37%,). Nearly two-thirds (54, 61%) of those with negative ANA had MSA/ MAA. Nearly half (18/54, 54.6%) had MSA associated with cytoplasmic patterns. ARS (anti-aminoacyl-tRNA synthetase) were associated with mechanic's hands (OR-7.06), ILD (OR-4.4), and arthritis (OR-2.23). Clinical associations of anti-Jo-1 and non-Jo-1 Anti synthetase syndrome (ASS) did not differ. Anti-MDA-5 associated with oral ulcers (OR-8.3), fever (OR-8.6) and weight loss (OR-7.35) in adults, and arthritis (OR-11.5), and periungual rash (OR-9.6) in children. Anti-TIF-1 gamma associated with photosensitivity (OR-10.44) and malignancy (OR-34) in adults, and cuticular overgrowth (OR-11.2) in children.Conclusion: Myositis autoantibodies are seen in two-thirds IlMs and are associated with distinct clinical subsets. Jo-1 and non-Jo-1 ASS exhibit similar characteristics. The association of anti-TIF1 gamma with malignancy was confirmed in adults. MSA/MAA were present in two-thirds of those with negative ANA and MSA were nearly always mutually exclusive. (C) 2020 Elsevier Inc. All rights reserved.