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Clinical diagnosis of pleuroparenchymal fibroelastosis

Cuneyt Tetikkurt

Pleuroparenchymal Fibroelastosis (PPFE) is a rare interstitial lung disease characterised by fibrosis of the visceral pleura with fibroelastotic changes predominating in the sub pleural lung parenchyma involving the upper lobes. The disease has unique clinical, radiological, and pathologic manifestations. Diagnosis is a challenge for clinicians because it is an extremely rare disease, there is no agreed consensus, and tissue biopsy is required for final diagnosis. PPFE may display a relentlessly progressive clinical course leading to an irreversible respiratory failure. PPFE diagnosis may cause serious difficulties due to the presence of other accompanying interstitial lung diseases, and comorbid sequela of the disease itself such as respiratory failure that usually precludes the diagnostic interventions for tissue biopsy.
This review focuses on the clinical PPFE profile revealing epidemiologic, physiopathologic mechanisms, clinical, radiologic, and pathologic manifestations to reach a definite diagnosis exclusively relying on the clinical findings alone without a tissue biopsy that is frequently unachievable due to the comorbid complications of pleuroparenchymal fibroelastosis.