Secondary pleuroparenchymal fibro-elastosis with airway-centered fibro-elastosis and con-strictive bronchiolitis associated with agriculture dust exposure

Abstract

Pleuroparenchymal fibro-elastosis (PPFE) is a rare idiopathic interstitial pneumonia. Secondary forms of PPFE may occur in patients following lung- or bone marrow transplantation. Here we report a middle-aged woman who presented with dyspnea and cough. She had been working as a cleaning woman in an agriculture company and logistic firm. Chest HRCT was highly suggestive for PPFE. Histopathological examination of a surgical biopsy showed a pattern of PPFE with airway-centered fibro-elastosis, constrictive bronchiolitis and birefringent particles in the pathological areas only. Using transmission electron microscopy with X-ray diffraction (TEM/EDX) these particles were identified as quartz and silicates, both components generally found in soil. The present case illustrates that secondary PPFE may result from agricultural dust exposure and stresses the need for a careful workup of the occupational history.