Pleuroparenchymal fibroelastosis: review of 81 cases.

Background: Pleuroparenchymal fibroelastosis (PPFE) is an upper lobe - predominant interstitial pneumonia pattern that can be idiopathic or secondary and has unclear clinical characteristics, disease course, and prognostic factors. This study analyzed PPFE patient characteristics, identified mortality predictors, and compared disease progression between idiopathic and secondary PPFE.

Research design and methods: We retrospectively identified patients with PPFE and analyzed demographic, clinical, radiologic, and pathologic data. Linear regression mixed models were used to assess predictors of mortality and lung function decline.

Results: Among 81 patients identified, 73% were women, median age was 69.4 years, and 75% were nonsmokers. Idiopathic PPFE comprised 46% of cases; secondary PPFE was most commonly familial (40%) or autoimmune related (33%). Over a median follow-up of 777 days, 38% died; the 5-year survival rate was 53%. Mortality risk did not differ between idiopathic and secondary PPFE. Lower body mass index, lower forced vital capacity (FVC) at diagnosis, and smoking history predicted mortality. The decline in FVC was slower in idiopathic PPFE than secondary PPFE.

Conclusions: Idiopathic and secondary PPFE differ in FVC decline but not in mortality risk. Familial and autoimmune conditions are the most common secondary causes.