Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
Pneumology Department, Hospital Universitario Vall d'Hebrón, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain.
Sci Rep. 2022 Mar 28;12(1):5289. doi: 10.1038/s41598-022-09353-z.
To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment.
为了评估患有肺动脉高压（PAH-SSc）和无肺动脉高压（非-PAH-SSc）的系统性硬化症（SSc）患者的严重程度标志物和结局，以及间质性肺病（ILD）对 PAH-SSc 的影响，我们纳入了来自西班牙 SSc 登记处的非-PAH-SSc 患者和来自西班牙 PAH 登记处的 PAH-SSc 患者。共纳入 364 例 PAH-SSc 患者和 1589 例非-PAH-SSc 患者。PAH-SSc 患者的纽约心脏病协会（NYHA）心功能分级（NYHA-FC）更差、用力肺活量（FVC）更差（81.2±20.6% vs 93.6±20.6%，P<0.001）、三尖瓣环平面收缩期位移（TAPSE）更差（17.4±5.2mm vs 19.9±6.7mm，P<0.001）、心包积液发生率更高（30% vs 5.2%，P<0.001），ILD 的患病率相似（41.8% vs. 44.9%）。在 PAH-SSc 患者中，ILD 与更差的血流动力学和肺功能测试（PFT）相关。ILD 患者和无 ILD 患者分别有 59.8%和 61.7%接受了一线联合治疗。PAH-SSc 患者的 5 年无移植生存率为 41.1%，而非-PAH-SSc 患者为 93.9%（P<0.001）。ILD 的严重程度不影响 PAH-SSc 患者的总体生存率。PAH-SSc 患者的多变量生存分析证实，诊断时的年龄、更差的 NYHA-FC、增加的肺动脉阻力、降低的弥散量、以及一线联合治疗是主要的危险因素。总之，在 PAH-SSc 患者中，临床、PFT 和血流动力学因素大大增加了死亡风险，而一线联合治疗则降低了死亡风险。并发的 ILD 会使 PAH-SSc 患者的血流动力学和 PFT 恶化，但不会影响生存率，无论 FVC 受损如何。
