Transbronchial cryobiopsy for diagnosis of pleuroparenchymal fibroelastosis

Rationale: Pleuroparenchymal fibroelastosis (PPFE) is a rare subtype of idiopathic interstitial pneumonias. PPFE mostly affects the upper lung zones and is characterized radiologically by pleural and subpleural fibrotic thickening with a reticular pattern. There is no established treatment for PPFE but lung transplantation can be considered for advanced stage. The gold standard for the diagnosis of PPFE is surgical lung biopsy (SLB) but the bronchoscopic transbronchial cryobiopsy (TBCB) is a less invasive alternative. Patient concerns: We report here two cases in which the diagnosis of PPFE was established with the help of TBCB. Diagnosis and interventions: Bronchoscopy with TBCB was performed under sedation with spontaneous ventilation and the help of an uncuffed ET tube. Outcomes: Histopathology showed intra-alveolar fibroblastic proliferation with elastosis, which confirmed the diagnosis of PPFE. Lessons: The current report demonstrates that TBCB can be a useful and safe tool to confirm the diagnosis of PPFE. According to our knowledge, this is one of few reports that shows successful diagnosis of PPFE by TBCB.