C39-37 Rapid Pulmonary Function Decline Is Associated With Poor Prognosis in Patients With Pleuroparenchymal Fibroelastosis

Abstract Background Pleuroparenchymal fibroelastasis (PPFE) is rare pulmonary disease with unique clinical. radiological, and pathological characteristics. Once regarded as a slowly progressing entity, it is now acknowledged that some patients with PPFE follow an rapidly progressive course that culminates in irreversible respiratory failure and early death. Objective The aim of this study was to evaluate the clinical, functional. radiological, and pathological characteristics in patients with PPFE with different diseases course. Mehtod This study was retrospective collection and evaluation the clinical, functional, radiological, and pahtological findings in patients with PPFE with idiopathic, and non-idiopathic disease course. All patients have been admitted to the pulmonary and critical care department of university teaching hospital from November 2014 to December 2024 Results We have enrolled 51 patients with PPFE diagnosed on results of clinical, radiological, and pahological findings. Depends on risk factors all patients have been divided into two groups: 1) 21 patients with idiopathic PPFE, with unknown risk factor; 2) 30 patients with non-idiopathic PPFE, with known risk factors (infective, connective tissue disease, familial and others). The age of patients was differand idiopathic PPFE oatients were older (p 0. 001), and male predominant (p 0. 001). Dyspnea on exertion and nonproductive cough are most common in patients with idiopathic PPFE (1. 80. 68-3. 02;p0. 02). Weight loss also was most frequent in idiopathic PPFE (p 0. 01), and often was accompanied by malnutrition and sarcopenia. Pulmonary function tests have been reveal a marked reduction in FVC in idiopathic PPFE patients (p 0. 004) and more rapidly decline of FVC in this group patients was indicated to the disease progression (2. 440. 84-5. 12; p0. 002). Annual decline of FVC in idiopathic PPFE was 280+₆8ml/year vs decline of non-idiopathic PPFE 135+₅4ml; (p 0. 003). Diffusion capacity for carbon monoxide (DLco), reflecting compromised alveolar-capillary gas exchange, was significantly decreased in idiopathic PPFE (p 0. 001). In idiopathic PPFE patients was identified more rapidly progression of radilogical findings included increased subpleural consolidation, massive pleural thickening, worsening upper lobe volume loss, and more extensive lower lobe interstitial involvement (p 0. 002). Pneumothorax was common in idiopathis PPFE patients (p 0. 01). Respiratory failure was more severe in idiopathic PPFE patients (p 0. 003) and severe hypoxemia with hypercapnia was common in advanced idiopathic PPFE patients (2. 72. 92-4. 86; p0. 002). Conclusions Idiopathic PPFE patients is characterized with more rapidly progressive disease course and have more rapidly pulmonary function decline, more rapidly progression of radiological abnormalities, and irreversible respiratory failure. Early identification and management of systemic effects such as malnutrition and complications such as pneumothorax are crucial for improving patient outcomes. This abstract is funded by: None