BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a common subtype of interstitial lung disease (ILD). Information about the associated comorbidities and predictors of survival among Saudi patients with IPF is limited.
AIMS: The aim of the study was to determine the clinical characteristics, associated comorbidities, and prognostic factors that impact IPF survival.
METHODS: Consecutive IPF patients diagnosed in our ILD center were included. The information analyzed included demographics, physiological parameters, and associated comorbidities, among others. Cox regression models were used to identify independent predictors of survival.
RESULTS: The data of 212 patients with IPF were available for the analysis. The mean age was 66.4 years, and 70.8% were male. The mean time between the onset of symptoms and diagnosis was 11.6 months (range: 1–48 months). Common comorbid conditions noted in the IPF cohort included pulmonary hypertension (49.6%), diabetes mellitus (43.2%), hypertension (42.2%), osteoporosis (40.4%), and gastroesophageal reflux disease (32.1%). Acute exacerbation (AE) was noted in 21.2% of the IPF patients. AE, final saturation <85%, walking distance <300 m, and antifibrotic therapy were independent predictors of survival.
CONCLUSIONS: In our IPF cohort, we found that there was a significant delay between the onset of symptoms and diagnosis. Moreover, we identified multiple comorbidities associated with IPF, which increases the burden on both IPF patients and clinicians. Importantly, AE and the use of antifibrotic therapy were independent predictors of survival. It is of paramount importance for clinicians to diagnose IPF at an early stage, refer patients to experienced centers, recognize comorbidities, and initiate antifibrotic therapy regardless of the underlying disease severity.


中文翻译：

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特发性肺纤维化患者的临床特征，合并症和预后


背景：特发性肺纤维化（IPF）是间质性肺病（ILD）的常见亚型。有关沙特IPF沙特患者的相关合并症和生存预测因素的信息有限。
目的：该研究的目的是确定影响IPF生存的临床特征，相关合并症和预后因素。
方法：包括在我们的ILD中心诊断出的连续IPF患者。分析的信息包括人口统计学，生理参数和相关合并症等。使用Cox回归模型来确定生存的独立预测因子。
结果：212例IPF患者的数据可供分析。平均年龄为66.4岁，男性为70.8％。从出现症状到诊断的平均时间为11.6个月（范围：1-48个月）。IPF队列中指出的常见合并症包括肺动脉高压（49.6％），糖尿病（43.2％），高血压（42.2％），骨质疏松症（40.4％）和胃食管反流病（32.1％）。在IPF患者中，有21.2％的患者出现急性加重（AE）。AE，最终饱和度<85％，步行距离<300 m和抗纤维化治疗是生存的独立预测因素。
结论：在我们的IPF队列中，我们发现症状发作和诊断之间存在明显的延迟。此外，我们发现了与IPF相关的多种合并症，这增加了IPF患者和临床医生的负担。重要的是，AE和抗纤维化疗法的使用是生存的独立预测指标。对于临床医生而言，尽早诊断IPF，将患者转诊至经验丰富的中心，认识合并症并开始抗纤维化治疗，无论其潜在的疾病严重程度如何，都是至关重要的。