Hwang Yun Jung, Lee Ju Kwang, Lee Jang Ho, Ahn Yura, Lee Sang Min, Hong Seokchan, Kim Ho Cheol
Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mokdong Hospital, College of Medicine, Ewha Womans University, Seoul, South Korea.
Sci Rep. 2025 Jul 9;15(1):24691. doi: 10.1038/s41598-025-08484-3.
Patients with systemic autoimmune rheumatic diseases-interstitial lung disease (SARD-ILD) exhibit diverse clinical courses, highlighting the importance of prognostic prediction for effective management. This study aimed to validate the gender-age-physiology (GAP) model in patients with SARD-ILD and identify additional prognostic factors. Clinical data of patients diagnosed with SARD-ILD at a tertiary center in South Korea were retrospectively analyzed. Using variables from the GAP model, along with exercise capacity, chest computed tomography (CT) patterns, and clinical course factors such as progressive pulmonary fibrosis (PPF), multivariate and survival analyses were performed to evaluate prognostic factors for 3-year mortality. Among 142 patients with SARD-ILD, 27 died and one underwent lung transplantation over a median follow-up period of 32.8 months. In the multivariate analysis, higher GAP stages, the combination of radiologic usual interstitial pneumonia (UIP) patterns and exertional hypoxia during the 6-min walk test (hazard ratio [HR]: 2.506, P = 0.049), and PPF (HR 3.481, P = 0.001) were independently associated with 3-year mortality. Additionally, among patients in advanced GAP groups (GAP stage II or III, n = 45), those with both exertional hypoxia and a UIP pattern had significantly worse prognosis (median survival: 18.0 vs. 31.2 months, P = 0.042). Additional prognostic factors beyond the GAP model were identified, including exercise capacity, CT imaging patterns, and clinical course. These findings underscore the importance of incorporating multiple prognostic indicators for improved stratification and management of patients with SARD-ILD.
系统性自身免疫性风湿疾病-间质性肺疾病（SARD-ILD）患者表现出多样的临床病程，这凸显了预后预测对有效管理的重要性。本研究旨在验证SARD-ILD患者的性别-年龄-生理学（GAP）模型，并确定其他预后因素。对韩国一家三级中心诊断为SARD-ILD的患者的临床数据进行了回顾性分析。使用GAP模型中的变量，以及运动能力、胸部计算机断层扫描（CT）模式和进行性肺纤维化（PPF）等临床病程因素，进行多变量和生存分析以评估3年死亡率的预后因素。在142例SARD-ILD患者中，中位随访期为32.8个月，27例死亡，1例接受了肺移植。在多变量分析中，较高的GAP分期、放射学普通型间质性肺炎（UIP）模式与6分钟步行试验期间运动性低氧血症的组合（风险比[HR]：2.506，P = 0.049）以及PPF（HR 3.481，P = 0.001）与3年死亡率独立相关。此外，在晚期GAP组（GAP分期II或III，n = 45）的患者中，同时具有运动性低氧血症和UIP模式的患者预后明显更差（中位生存期：18.0对31.2个月，P = 0.042）。确定了GAP模型之外的其他预后因素，包括运动能力、CT成像模式和临床病程。这些发现强调了纳入多个预后指标以改善SARD-ILD患者分层和管理的重要性。
