Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, and pathological PPFE is also observed in secondary interstitial pneumonia. This study aimed to evaluate the pathological findings associated with radiological PPFE-like lesions and the clinical and morphological features of patients with pathological PPFE.

We retrospectively reviewed the pathology of the explanted lungs from 59 lung transplant recipients with radiological PPFE-like lesions. Pathological PPFE lesions were identified in 14 patients with idiopathic cases and 12 patients with secondary cases. Pathological PPFE was associated with previous pneumothorax, a volume loss in the upper lobes and a flattened chest. Patients with idiopathic and secondary cases with pathological PPFE had similar clinical, radiological, and pathological findings, while fibroblastic foci were more common in patients with idiopathic cases, and patients with secondary cases more frequently showed alveolar septal thickening with elastosis or fibrosis. Post-transplantation survival did not differ between patients with idiopathic and secondary cases with pathological PPFE (log-rank; P=0.57) and was similar between patients with idiopathic cases with pathological PPFE and patients with idiopathic pulmonary fibrosis (IPF) (log-rank; P = 0.62).

Not all patients with interstitial pneumonia with radiological PPFE-like lesions have pathological PPFE. Characteristic clinical features can suggest the presence of pathological PPFE, and idiopathic and secondary cases with pathological PPFE are similar except for fibroblastic foci in idiopathic cases and alveolar septal thickening with elastosis or fibrosis in secondary cases. Patients with pathological PPFE have a similar prognosis to those with IPF after transplantation.