Cha YJ, et al. – The clinical, radiologic, and pathologic features of patients with histologically proven pleuroparenchymal fibroelastosis (PPFE) were investigated in this study. According to the findings obtained, PPFE appeared to be a histologic finding that might partly overlap with other chronic lung diseases. There can be various underlying clinical states.

Methods

• For this study, 7 patients who had histologic features of PPFE that were definitive or consistent with PPFE were incorporated.
• After that, clinical data and radiologic findings of chest computed tomography were obtained.
• Finally, histologic evaluation was performed by 2 pathologists.

Results

• The results of this study showed that 4 patients had elevated serum autoantibodies, 3 had a history of organ transplantation, and 1 had acute Aspergillus infection.
• It was observed in the findings that all patients had various degrees of restrictive patterns on pulmonary function tests.
• Histologic examination showed a variable extent of PPFE.
• Fibroblastic foci, patchy lymphocytic infiltration, and pneumothorax were seen, but these findings were nonspecific.
• Radiologically, PPFE lesions were distributed diffusely, as opposed to being limited to the upper lungs.