Pleuroparenchymal Fibroelastosis: A Case Report

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare, idiopathic interstitial pneumonia. wherein the first symptom might be dyspnea or a dry cough. The condition can also be manifested with chest pain secondary to pneumothorax. While the definitive diagnosis is based on a histological evaluation (which is not often performed), a computed tomography scan shows findings, such as apical fibrosis and pleural thickening of the apical lobes, which help assess the diagnosis. We describe a case of PPFE diagnosed radiologically in a 69-year-old man. This case highlights that PPFE is a pathology that can go unnoticed for a long time, and patients might neglect the revealing symptoms such as coughing.

Pleuroparenchymal Fibroelastosis. Is it Really Uncommon? Report of Eight Cases

“…It has also been proposed that chronic inflammation secondary to recurrent bronchopulmonary infections would account for the presence of fibrosis. 3 , 4 …”

“…The most typical clinical manifestations are cough, dyspnea, pneumothorax, and a low body mass index. 4 Radiological lesions may precede symptomatology by months/years. 5 In early stages, CT usually shows increased biapical subpleural density, which later develops into pleural thickening, with subpleural fibrosis and bronchiectasis/bullae.…”

“… 5 In early stages, CT usually shows increased biapical subpleural density, which later develops into pleural thickening, with subpleural fibrosis and bronchiectasis/bullae. 4 Other possible findings are: decreased anteroposterior and transverse diameter of the thorax, hilar adenopathy or a reticulonodular pattern. In up to 75% of cases, radiological lesions of PPFE coexist with those of other entities (e.g., IPF, tuberculosis, systemic sclerosis).…”

“… 2 Furthermore, due to the apical location, confusion with tuberculosis may arise, although unilateral involvement would be more indicative of the latter. 3 , 4 , 5 An increase in the RV/TLC ratio is characteristic, which is explained because apical fibrosis causes collapse, with compensatory bibasal hyperinflation. 2 The most notable histopathological findings are visceral pleural fibrosis and subpleural fibroelastosis.…”