Clinical Diagnosis of Pleuroparenchymal Fibroelastosis

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is an extremely rare interstitial lung disease (ILD) characterised
by fibroelastotic changes predominating in the subpleural lung parenchyma with visceral pleural fibrosis
involving the upper lobes. It has distinctive clinical, radiological, and pathological manifestations.
Diagnosis constitutes literally a stalemate because of its rarity, unavailability of an agreed diagnostic
consensus, and requirement of tissue biopsy for an accurate final diagnosis that can not be performed in
most of the patients owing to the comorbid complications of the disease itself. Identification of PPFE
may also pose great difficulties due to the coexistence of other interstitial lung diseases. PPFE usually
exhibits a persistently deteriorating prognostic course culminating in fatal complications including
respiratory failure, pulmonary hypertension, cor pulmonale, or pneumothorax. Presence of disease
relevant complications frequently preclude invasive tissue biopsy interventions in these patients leading
to a diagnostic challenge for clinicians.
This review aims to provide a definitive diagnosis based on entirely the clinical manifestatins of PPFE
by shedding light on the pathogenesis, clinical, and radiologic findings of the disease. Histopathological
tissue evaluation was also included in the diagnostic approach for patients whose condition was suitable
for an invasive biopsy intervention. With this review, it is concluded that an approach consisting of
exclusively clinical manifestations will ensure adequate support for an accurate PPFE diagnosis without
any requirement for histopathological tissue examination.