ArticleTherapeutic advances in respiratory disease
Pleuroparenchymal fibroelastosis from a rheumatologic perspective.
Article in Therapeutic advances in respiratory disease. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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6 authors.
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Abstract
backgroundPleuroparenchymal 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.
objectivesTo describe demographics, imaging patterns, and outcomes in SARD-associated PPFE.
methodsPatients 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.
resultsFifteen 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.
conclusionSystemic 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.
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