Department of Internal Medicine and Rheumatology, Dr. Ion Cantacuzino Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Ann Rheum Dis. 2018 Jan;77(1):128-132. doi: 10.1136/annrheumdis-2017-211480. Epub 2017 Oct 23.
Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc with early PAH (SSc-PAH) from the DETECT cohort.
Patients with SSc-PAH enrolled in DETECT were observed for up to 3 years. Associations between cross-sectional variables and disease progression (defined as the occurrence of any of the following events: WHO Functional Class worsening, combination therapy for PAH, hospitalisation or death) were analysed by univariable logistic regression.
Of 57 patients with PAH (median observation time 12.6 months), 25 (43.9%) had disease progression. The following factors (OR (95% CI)) were associated with disease progression: male gender (4.1 (1.2 to 14.1)), high forced vital capacity % predicted/carbon monoxide lung diffusion capacity (DLCO)% predicted ratio (3.6 (1.2 to 10.7)), high Borg Dyspnoea Index (1.7 (1.1 to 2.6)) and low DLCO% predicted (non-linear relationship).
More than 40% of early-diagnosed patients with SSc-PAH had disease progression during a short follow-up time, with male gender, functional capacity and pulmonary function tests at PAH diagnosis being associated with progression. This suggests that even mild PAH should be considered a high-risk complication of SSc.
肺动脉高压（PAH）是系统性硬皮病（SSc）的严重并发症。在这项纵向研究中，我们旨在从 DETECT 队列中识别与早期 SSc-PAH 患者不良预后相关的因素。
在 DETECT 中招募的 SSc-PAH 患者接受了长达 3 年的观察。通过单变量逻辑回归分析了横断面变量与疾病进展（定义为出现以下任何事件：WHO 功能分类恶化、PAH 联合治疗、住院或死亡）之间的关联。
在 57 例 PAH 患者中（中位观察时间为 12.6 个月），有 25 例（43.9%）发生疾病进展。以下因素（OR（95%CI））与疾病进展相关：男性（4.1（1.2 至 14.1））、用力肺活量预测值/一氧化碳弥散量（DLCO）%预测值比值高（3.6（1.2 至 10.7））、Borg 呼吸困难指数高（1.7（1.1 至 2.6））和 DLCO%预测值低（非线性关系）。
在短时间的随访中，超过 40%的早期诊断为 SSc-PAH 的患者发生了疾病进展，男性、功能能力和 PAH 诊断时的肺功能测试与进展相关。这表明，即使是轻度 PAH 也应被视为 SSc 的高风险并发症。
