A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies
A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies
复制标题
DOI：
10.1093/rheumatology/kex021
复制
发表时间：
2017-06-01
期刊：
影响因子：
5.5
通讯作者：
Danoff, Sonye K.
中科院分区：
文献类型：
--
作者：
Pinal-Fernandez, Iago;Casal-Dominguez, Maria;Danoff, Sonye K.
目标。目的探讨不同抗合成酶综合征（ASyS）自身抗体患者的患病率、出现率及临床特征的严重程度。所有约翰霍普金斯大学的肌炎纵向队列受试者均为ASyS自身抗体阳性。前瞻性收集临床信息，包括症状、体征、强度、肌酸激酶浓度和肺功能测试。采用单因素和多因素分析评估标准化死亡率和肿瘤发生率以及不同器官表现的出现率和强度，并比较ASyS自身抗体。抗jo1阳性124例（73.4%），抗pl12阳性23例（13.6%），抗pl7阳性16例（9.5%），抗ej或抗oj阳性3例（1.8%）。平均随访时间为4.1年。Anti-PL12在黑人受试者中更为常见。抗pl12和抗pl7与更普遍和严重的肺部受累相关，通常没有肌肉受累。与抗pl12患者相比，抗jo1患者表现出更严重的肌肉受累。并发抗ro52在抗jo1患者中更为普遍，并且与机械性手的早期发展、dm特异性皮肤表现和关节炎有关。与ASyS抗体状态无关，黑人患者表现出比白人患者更严重的肺部受累。与美国普通人群相比，ASyS患者的死亡率或癌症风险没有显著增加。不同的ASyS自身抗体与ASyS谱系中表型不同的亚群相关。抗pl7和抗pl12的特点是更严重的肺部受累，而抗jo1的特点是更严重的肌肉受累。黑人是与肺部疾病严重程度相关的主要预后因素。
Objective. The aim was to study the prevalence, rate of appearance and severity of clinical features in patients with different anti-synthetase syndrome (ASyS) autoantibodies.Methods. All Johns Hopkins Myositis Longitudinal Cohort subjects positive for any ASyS autoantibodies were included. Clinical information, including symptoms, signs, strength, creatine kinase concentrations and pulmonary function tests, were prospectively collected. The standardized mortality and cancer rates and the rate of appearance and intensity of the different organ manifestations were assessed using univariate and multivariate analysis and compared between ASyS autoantibodies.Results. One hundred and twenty-four (73.4%) patients were positive for anti-Jo1, 23 (13.6%) for anti-PL12, 16 for anti-PL7 (9.5%) and 3 (1.8%) for anti-EJ or anti-OJ, respectively. The mean length of follow-up was 4.1 years. Anti-PL12 was more frequent in black subjects. Anti-PL12 and anti-PL7 were associated with more prevalent and severe lung involvement, often without muscle involvement. Anti-Jo1 displayed more severe muscle involvement compared with anti-PL12 patients. Concurrent anti-Ro52 was more prevalent in anti-Jo1 patients and was associated with earlier development of mechanic's hands, DM-specific skin findings and arthritis. Independent of ASyS antibody status, black patients demonstrated more severe lung involvement than white patients. There was no significant increase in mortality or cancer risk in ASyS patients compared with the general US population.Conclusion. Different ASyS autoantibodies are associated with phenotypically distinct subgroups within the ASyS spectrum. Anti-PL7 and anti-PL12 are characterized by more severe lung involvement, whereas anti-Jo1 is associated with more severe muscle involvement. Black race is a major prognostic factor associated with lung disease severity.