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Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes.

Reddy, TL; Tominaga, M; Hansell, DM; von der Thusen, J; Rassl, D; Parfrey, H; Guy, S; ... Nicholson, AG; + view all (2012) Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J , 40 (2) pp. 377-385. 10.1183/09031936.00165111.

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Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterised by predominantly upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls. The aim of this study was to review cases fulfilling published imaging and histological criteria, and identify any common clinical features that may suggest an underlying aetiology for a condition that has previously been regarded as idiopathic. Of 12 patients (seven females, median age 57 yrs), the presenting symptoms were shortness of breath (11 out of 12 patients) and dry cough (six out of 12 patients). Seven patients reported recurrent infections during the course of their disease. Five demonstrated nonspecific autoantibody positivity. Two patients had a family history of interstitial lung disease (ILD). High-resolution computed tomography features of lung disease remote from the pleuroparenchymal changes were present in six out of 12 patients (coexistent fibrosis, n=5; bronchiectasis, n=1). Of seven patients with tissue sampled from the lower lobes, four patients showed less intense PPFE changes (one with additional features of hypersensitivity pneumonitis) and three showed usual interstitial pneumonia. PPFE is a distinct clinicopathological entity, with clinical data suggesting a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms may also contribute to the development of these changes. PPFE may also present with more diffuse involvement than previously reported, and coexist with different patterns of ILD.

Type: Article
Title: Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes.
Location: England
DOI: 10.1183/09031936.00165111
Keywords: Adult, Aged, Aged, 80 and over, Biopsy, Cough, Dyspnea, Female, Humans, Lung, Lung Diseases, Interstitial, Male, Middle Aged, Phenotype, Pleura, Pulmonary Alveoli, Pulmonary Fibrosis, Tomography, X-Ray Computed, Treatment Outcome
URI: http://discovery.ucl.ac.uk/id/eprint/1344046
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