Pleuroparenchymal fibroelastosis: report of two cases in Brazil

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause.

Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?

“…As a rule, PPFE is associated with a restrictive functional deficit and an impairment of CO diffusion capacity (DLCO) [5], which are considered the principal prognostic biomarkers of disease progression [17,18]. Radiological features include pleural fibrotic thickening and subpleural fibroelastosis, with an upper lobe predominance associated with traction bronchiectasis, architectural distortion, loss of volume and reticulations [[17], [18], [19], [20], [21], [22]]. In advanced stages of disease, the architectural distortion can involve also adjacent and lower lobes.…”

“…Histopathologic features include aggregates of elastic fibers, especially in the sub-pleural areas, and intra-alveolar collagenous fibrosis with septal elastosis (with or without collagenous thickening of the visceral pleura) [20,22]. The interface with uninvolved parenchyma is typically abrupt; the architectural distortion mainly occurs in sub-pleural areas but a peri-bronchial distribution has also been reported [21]. PPFE diagnostic criteria have been postulated only 2 years ago and include the detection of multilobar subpleural and/or centrilobular fibrous interstitial pneumonia with an extensive (>80%) proliferation of elastic fibers in non-atelectasis lung [22].…”

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

“…Histopathological features of PPFE include aggregates of elastic fibers, especially in subpleural areas, and intra-alveolar collagenous fibrosis with septal elastosis (with or without collagenous thickening of the visceral pleura) [20,22]. The interface with unaffected parenchyma is typically abrupt; architectural distortion mainly occurs in subpleural areas although peribronchial distribution can also be observed [21]. Two years ago, the following diagnostic criteria were proposed for PPFE: multilobar subpleural and/or centrilobular fibrous interstitial pneumonia with extensive proliferation of elastic fibers in nonatelectatic lung areas, along with absent-to-mild chronic inflammation and absent-to-rare granulomas [22].…”