Fibroelastosi pleuroparenchimale familiare diagnosticata mediante criobiopsia polmonare

Idiopathic pleuroparenchymal fibroelastosis (PPFE), recently included in the updated international classification of idiopathic interstitial pneumonias (IIPs), is a rare condition characterized by pleural fibrosis and subadjacent parenchymal fibroelastosis, with an upper-zone predominance. It might also occur in the context of familial IIPs or be associated to pre-existing underlying conditions, such as recurrent infections, transplantations, previous chemotherapies, and connective tissue diseases. Although a definite consensus regarding the diagnostic criteria has yet to be established, a proper diagnostic work-up necessarily requires the identification of morphological features consistent with PPFE. However, surgical lung biopsy is characterized by appreciable costs and risks, and many subjects cannot undergo the procedure because of an increased risk of perioperative morbidity and mortality, due to advanced age, severity of disease, and comorbidities. In this context, transbronchial lung criobiopsy has been recently proposed as a valuable alternative tool for the histopathologic assessment of diffuse parenchymal lung diseases, showing a high diagnostic yield with an excellent safety profile. Here, we report a case of a young woman with familial history of idiopathic pulmonary fibrosis and rheumatoid arthritis, diagnosed with PPFE through transbronchial lung criobiopsy.