Division of Rheumatology, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan; Division of General Medicine, Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Department of Life Sciences, National Chung Hsing University, Taichung, Taiwan; Department of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Yunlin, Taiwan.
Semin Arthritis Rheum. 2020 Oct;50(5):957-962. doi: 10.1016/j.semarthrit.2020.06.005. Epub 2020 Jun 16.
OBJECTIVE: To estimate and compare the incidence and survival impact of pulmonary arterial hypertension (PAH) among patients with various connective tissue diseases (CTDs). METHODS: We used a national health insurance database in Taiwan and enrolled patients with incident systemic sclerosis (SSc), systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA) between 2002 and 2013. We calculated the cumulative incidence of PAH and the probability of survival after PAH diagnosis by the Kaplan-Meier method. RESULTS: Of 1653 SSc patients, 127 (7.68%) developed PAH. Of 11,735 SLE patients, 242 (2.06%) developed PAH. Of 17,316 pSS patients, 1811 PM/DM patients, and 32,296 RA patients, 38 (0.22%), 6 (0.33%) and 15 (0.04%) patients developed PAH, respectively. The most common CTD among all CTD-PAH patients was SLE (57%), followed by SSc (30%), pSS (9%), RA (3%) and PM/DM (1%). The 1-, 3- and 5-year survival rates for SSc-PAH were 88.3%, 72.1% and 61.9%, respectively. The 1-, 3- and 5-year survival rates for SLE-PAH were 87.6%, 75.8% and 69.4%, respectively. The 1-, 3- and 5-year survival rates for pSS-PAH were 90.8%, 86.8% and 80.6%, respectively. CONCLUSIONS: SLE was the most common underlying CTD, followed by SSc, in the total CTD-PAH population. However, the highest risk of developing PAH was observed in the SSc groups. PAH is a very rare complication in pSS, PM/DM and RA. Patients with SSc-PAH have the worst survival rate compared to those with SLE-PAH or pSS-PAH.
目的：估计和比较各种结缔组织疾病（CTD）患者中肺动脉高压（PAH）的发病率和生存影响。
方法：我们使用台湾的全民健康保险数据库，招募了 2002 年至 2013 年间患有系统性硬化症（SSc）、系统性红斑狼疮（SLE）、原发性干燥综合征（pSS）、多发性肌炎/皮肌炎（PM/DM）和类风湿关节炎（RA）的患者。我们通过 Kaplan-Meier 法计算 PAH 的累积发病率和 PAH 诊断后的生存率。
结果：在 1653 名 SSc 患者中，有 127 名（7.68%）发展为 PAH。在 11735 名 SLE 患者中，有 242 名（2.06%）发展为 PAH。在 17316 名 pSS 患者、1811 名 PM/DM 患者和 32296 名 RA 患者中，分别有 38 名（0.22%）、6 名（0.33%）和 15 名（0.04%）患者发展为 PAH。所有 CTD-PAH 患者中最常见的 CTD 是 SLE（57%），其次是 SSc（30%）、pSS（9%）、RA（3%）和 PM/DM（1%）。SSc-PAH 的 1、3 和 5 年生存率分别为 88.3%、72.1%和 61.9%。SLE-PAH 的 1、3 和 5 年生存率分别为 87.6%、75.8%和 69.4%。pSS-PAH 的 1、3 和 5 年生存率分别为 90.8%、86.8%和 80.6%。
结论：SLE 是总 CTD-PAH 人群中最常见的基础 CTD，其次是 SSc。然而，PAH 发病风险最高的是 SSc 组。PAH 是 pSS、PM/DM 和 RA 中非常罕见的并发症。与 SLE-PAH 或 pSS-PAH 相比，SSc-PAH 患者的生存率最差。
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