Nagy Tamas, Toth Nora Melinda, Palmer Erik, Polivka Lorinc, Csoma Balazs, Nagy Alexandra, Eszes Noémi, Vincze Krisztina, Bárczi Enikő, Bohács Anikó, Tárnoki Ádám Domonkos, Tárnoki Dávid László, Nagy György, Kiss Emese, Maurovich-Horvát Pál, Müller Veronika
Department of Pulmonology, Semmelweis University, 1083 Budapest, Hungary.
Medical Imaging Centre, Semmelweis University, 1082 Budapest, Hungary.
Biomedicines. 2022 Aug 31;10(9):2129. doi: 10.3390/biomedicines10092129.
Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Progressive pulmonary fibrosis (PPF) is defined as progression in 2 domains including clinical, radiological or lung-function parameters. Our aim was to assess predictors of functional decline in SSc-ILD patients and compare disease behavior to that in idiopathic pulmonary fibrosis (IPF) patients. Patients with normal forced vital capacity (FVC > 80% predicted; SSc-ILD: n = 31; IPF: n = 53) were followed for at least 1 year. Predictors of functional decline including clinical symptoms, comorbidities, lung-function values, high-resolution CT pattern, and treatment data were analyzed. SSc-ILD patents were significantly younger (59.8 ± 13.1) and more often women (93 %) than IPF patients. The median yearly FVC decline was similar in both groups (SSc-ILD = −67.5 and IPF = −65.3 mL/year). A total of 11 SSc-ILD patients met the PPF criteria for functional deterioration, presenting an FVC decline of −153.9 mL/year. Cough and pulmonary hypertension were significant prognostic factors for SSc-ILD functional progression. SSc-ILD patients with normal initial spirometry presenting with cough and PH are at higher risk for showing progressive functional decline.
间质性肺疾病（ILD）是系统性硬化症（SSc）患者死亡的主要原因。进行性肺纤维化（PPF）被定义为在临床、放射学或肺功能参数这两个领域出现进展。我们的目的是评估SSc-ILD患者功能下降的预测因素，并将疾病行为与特发性肺纤维化（IPF）患者进行比较。对用力肺活量正常（FVC>预测值的80%；SSc-ILD：n = 31；IPF：n = 53）的患者进行了至少1年的随访。分析了包括临床症状、合并症、肺功能值、高分辨率CT模式和治疗数据等功能下降的预测因素。与IPF患者相比，SSc-ILD患者明显更年轻（59.8±13.1），女性比例更高（93%）。两组的年FVC下降中位数相似（SSc-ILD = -67.5，IPF = -65.3 mL/年）。共有11例SSc-ILD患者符合PPF功能恶化标准，FVC下降为-153.9 mL/年。咳嗽和肺动脉高压是SSc-ILD功能进展的重要预后因素。初始肺量计检查正常且伴有咳嗽和肺动脉高压的SSc-ILD患者出现功能进行性下降的风险更高。
