Objective. To compare the clinical characteristics and identify the longterm outcomes of Chinese patients with different antisynthetase antibodies. Methods. We investigated retrospectively 124 consecutive patients with antisynthetase syndrome. Medical records, laboratory results, and computed tomography images were obtained. Results. The antisynthetase antibodies we investigated were anti-Jo1 (n = 62), anti-PL7 (n = 31), anti-PL12 (n = 12), and anti-EJ (n = 19). The overall prevalence of interstitial lung disease (ILD) reached 94.4% among study patients. Eleven patients (8.9%) developed rapidly progressive ILD (RP-ILD). Eight patients (6.5%) experienced malignancy. RP-ILD was statistically more prevalent in patients with antisynthetase syndrome with anti-PL7 than those without anti-PL7 (p = 0.028). Anti-Ro52-positive patients with antisynthetase syndrome experienced higher frequency of RP-ILD than those without anti-Ro52 (p = 0.001). Further, anti-PL7-positive patients coexisting with anti-Ro52 exhibited more RP-ILD than those without anti-Ro52 (p = 0.001). Patients with antisynthetase syndrome with RP-ILD had a higher proportion of neutrophils in bronchoalveolar lavage fluid and serum ferritin than those without RP-ILD (p = 0.006 and p = 0.013, respectively). Although no differences were observed between the Kaplan-Meier curves of the 4 antisynthetase antibodies subgroups (p = 0.349), the survival rate of patients with anti-PL7 decreased more rapidly in the early stage of longterm followup compared with those with other antisynthetase antibodies. The presence of RP-ILD, malignancy, and elevated serum ferritin was identified to be associated with poor prognosis in patients with antisynthetase syndrome. Conclusion. Our study investigates the clinical phenotypes and outcomes of patients with antisynthetase syndrome with distinct antisynthetase antibodies and highlights the link between the anti-PL7 antibody and RP-ILD.
基金:
National Natural Science Foundation of ChinaNational Natural Science Foundation of China (NSFC) [91542121, 81571603]; Capital Foundation of Medical Developments [2016-2-4063]; Science and Technology Commission Foundation of Beijing [Z151100004015143]
第一作者单位:[1]Peking University China-Japan Friendship School of Clinical Medicine
通讯作者:
通讯机构:[1]Peking University China-Japan Friendship School of Clinical Medicine[2]Department of Rheumatology, China-Japan Friendship Hospital[*1]Department of Rheumatology, China-Japan Friendship Hospital, Beijing 100029, China
推荐引用方式(GB/T 7714):
Shi Jingli,Li Shanshan,Yang Hanbo,et al.Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies[J].JOURNAL of RHEUMATOLOGY.2017,44(7):1051-1057.doi:10.3899/jrheum.161480.
APA:
Shi, Jingli,Li, Shanshan,Yang, Hanbo,Zhang, Yamei,Peng, Qinglin...&Wang, Guochun.(2017).Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies.JOURNAL of RHEUMATOLOGY,44,(7)
MLA:
Shi, Jingli,et al."Clinical Profiles and Prognosis of Patients with Distinct Antisynthetase Autoantibodies".JOURNAL of RHEUMATOLOGY 44..7(2017):1051-1057