Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort.
Mark P SteeleAnna L PeljtoSusan K MathaiStephen M HumphriesTami J BangAndrea OhShawn TeagueGiuseppe CicchettiChristopher SigakisJonathan A KropskiJames E LoydTimothy S BlackwellKevin K BrownMarvin I SchwarzRachel A WarrenJulia PowersAvram D WaltsCheryl MarkinTasha E FingerlinIvana V YangDavid A LynchJoyce S LeeDavid A SchwartzPublished in: American journal of respiratory and critical care medicine (2022)
The incidence of PrePF in this at-risk population is at least 100-fold higher than that reported for sporadic idiopathic pulmonary fibrosis (IPF). While PrePF and IPF represent distinct entities, our study demonstrates that PrePF, like IPF, is progressive and associated with decreased survival.