Pleuroparenchymal fibroelastosis – a new diagnostic and therapeutic challenge for pneumologists

Introduction:

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition with upper lobe predominant fibroelastosis and pleural thickening. PPFE is now recognized as specific rare entity, usually idiopathic, and included as a new entity in the updated classification of the idiopathic interstitial pneumonia.

Case Report:

A 63 years old non-smoking woman presented with progressive dyspnoea on exertion and dry cough. There was no significant occupational exposure, allergy or history of systemic disease. 3 years ago idiopathic pulmonary fibrosis (IPF) was diagnosed by surgical biopsy in another hospital.

At current hospitalization physical examination revealed bilateral crackles but no clubbing. High resolution computed tomography (HR-CT) demonstrated upper lobe predominant pleural thickening and reticular opacities, traction bronchiectasis, subpleural and peribronchial consolidation, upper lobe shrinkage, architectural distortion, and minor honeycombing in the lower lobes. Bronchoalveolar lavage cell differential was normal. Forced vital capacity was markedly lower (55% of predicted) than 3 years ago (72% of predicted). There was no clinical or serological evidence of a connective tissue disease, vasculitis or hypersensitivity pneumonitis.

Because of the atypical location of fibrosis in HR-CT, the surgical biopsy of 2009 was reevaluated. Histologically, subpleural fibroelastosis, alveolar-septal fibrosis and a possible usual interstitial pneumonia (UIP) pattern (absence of fibroblastic foci) were found.

Conclusion:

In the case of an atypical UIP pattern on imaging with upper lobe predominant fibrosis and pleural thickening, PPFE should be considered as a differential diagnosis. The clinical presentation may be indistinguishable from IPF.