Idiopathic pleuroparenchymal fibroelastosis: Possibility of making a diagnosis without surgical lung biopsy

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare interstitial pneumonia in idiopathic interstitial pneumonias (IIPs). Currently, surgical lung biopsy (SLB) is needed to make a diagnosis for IPPFE. But it is often difficult to perform SLB for the patients who were clinically and radiologically suspected IPPFE. Here we studied the patients with 1) unknown etiology (Idiopathic), 2) intense pleural thickening with upper lobe predominace on high-resolution computed tomography (HRCT), and 3) disease progression. Among 20 patients fulfilling the above criteria from 2004 to 2013 in our facility and related hospitals, six patients had surgical lung biopsy. Biopsy specimens from all six patients were consistent with pathological diagnosis of IPPFE. There was no significant difference in clinical characteristics and in survival between the patients with or without biopsy. The clinical features of all patients are relatively uniform, including older age, low BMI, restrictive pattern in pulmonary function test, and they had very poor survival (3-yr survival; 38.4%). Collectively, patients fulfilling above criteria were highly suspected of IPPFE. Their clinical characteristics were unique, and the prognosis was poor.