Liu Z, Yang X, Tian Z, Qian J, Wang Q, Zhao J, Huang C, Liu Y, Guo X, Wang H, Lai J, Li M, Zhao Y, Zeng X
1 Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
2 Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Lupus. 2018 Jun;27(7):1072-1080. doi: 10.1177/0961203318756287. Epub 2018 Feb 8.
Pulmonary arterial hypertension (PAH) is a rare and severe complication of primary Sjögren's syndrome (pSS). Cohort studies indicate that the underlying diseases of PAH associated with connective tissue disease (CTD-PAH) in Asian countries are different from that in the United States and in Europe. This study investigated the clinical characteristics, survival, and prognostic factors of pSS-PAH in Chinese patients. We enrolled 29 patients with pSS-PAH who visited our referral center during August 2007 and May 2015. PAH was confirmed by right heart catheterization (RHC). Baseline demographic data, clinical manifestations, laboratory tests, autoantibody results, hemodynamics data, and treatment regimens were analyzed. All patients were followed up at the Department of Rheumatology, Peking Union Medical College Hospital (PUMCH) every three to six months. All patients were female. Mean age of pSS onset was 34.4 ± 11.1 years. Mean age of PAH diagnosed by RHC was 40.6 ± 9.0 years. There was no significant difference in age, disease duration, and hemodynamic findings between the survivors and the non-survivors in this cohort. The overall one-, three-, and five-year survival rates were 80.2%, 74.8%, and 67.4%, respectively. Prognostic factors of mortality were time between pSS onset and PAH onset (HR 1.102, 95% CI 1.017-1.185, p = 0.018) and cardiac index < 2 l/min/m (HR 5.497, 95% CI 1.063-28.434, p = 0.042). In contrast, the use of immunosuppressants (HR 0.110, 95% CI 0.024-0.495, p = 0.004) was related to better survival. This study demonstrates that the predictors of mortality in patients with pSS-PAH include delayed onset of PAH in pSS and worse cardiac function. Immunosuppressants can improve the prognosis of pSS-PAH.
肺动脉高压（PAH）是原发性干燥综合征（pSS）一种罕见且严重的并发症。队列研究表明，亚洲国家与结缔组织病相关的PAH（CTD-PAH）的基础疾病与美国和欧洲不同。本研究调查了中国pSS-PAH患者的临床特征、生存率及预后因素。我们纳入了2007年8月至2015年5月期间就诊于我们转诊中心的29例pSS-PAH患者。PAH通过右心导管检查（RHC）确诊。分析了基线人口统计学数据、临床表现、实验室检查、自身抗体结果、血流动力学数据及治疗方案。所有患者在北京协和医院（PUMCH）风湿免疫科每三到六个月随访一次。所有患者均为女性。pSS发病的平均年龄为34.4±11.1岁。通过RHC诊断PAH的平均年龄为40.6±9.0岁。该队列中幸存者与非幸存者在年龄、病程及血流动力学结果方面无显著差异。总体1年、3年和5年生存率分别为80.2%、74.8%和67.4%。死亡的预后因素为pSS发病至PAH发病的时间（HR 1.102，95%CI 1.017-1.185，p = 0.018）及心脏指数<2 l/min/m²（HR 5.497，95%CI 1.063-28.434, p = 0.042）。相反，使用免疫抑制剂（HR 0.110，95%CI 0.024-0.495，p = 0.004）与更好的生存率相关。本研究表明，pSS-PAH患者死亡的预测因素包括pSS中PAH发病延迟及心脏功能较差。免疫抑制剂可改善pSS-PAH的预后。
