Pleuroparenchymal fibroelastosis from a rheumatologic perspective

BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) associated with systemic autoimmune rheumatic diseases (SARDs). Its clinical characteristics and outcomes in SARDs remain poorly defined. OBJECTIVES: To describe demographics, imaging patterns, and outcomes in SARD-associated PPFE. METHODS: Patients diagnosed with PPFE and SARDs at Mayo Clinic (2000-2024) were included. PPFE diagnosis was confirmed by high-resolution computed tomography, and SARD diagnoses were established by rheumatologists. RESULTS: Fifteen of 62 patients with PPFE had SARDs with majority having systemic sclerosis (SSc)(n= 7, 46%). Median age at PPFE diagnosis was 57.2 years; 80% were female; 40% were ever-smokers; 40% required supplemental oxygen; 5 had pulmonary hypertension. Coexisting ILD patterns included UIP (26.7%) and NSIP (20%). Coexisting ILD patterns included usual interstitial pneumonia (26.7%) and nonspecific interstitial pneumonia (20%). Three patients died, including two with SSc. CONCLUSION: Systemic sclerosis was the most frequently observed SARD among patients with PPFE, and may coexist with other ILD patterns; recognition may inform risk stratification, multidisciplinary evaluation, and longitudinal follow-up.