A Not-So-Stray Disease: Pleuroparenchymal Fibroelastosis, a Challenging Diagnosis
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Pleuroparenchymal fibroelastosis (PPFE) is a rare disease within the spectrum of diffuse interstitial lung diseases, classified as a rare idiopathic interstitial pneumonia by the American Thoracic Society (ATS) and the European Respiratory Society (ERS). Histologically, it is characterized by fibrosis of the visceral pleura associated with fibroelastic changes in the subpleural lung parenchyma, predominantly in the upper lobes. Its real incidence is unknown, due to underdiagnosis and the lack of standardized diagnostic criteria. This article reports two clinical cases diagnosed with advanced PPFE in a medical center in Montevideo, Uruguay. This condition presents a diagnostic challenge, and its early recognition is crucial as it might be more prevalent than perceived. PPFE should be suspected in patients with pleural thickening associated with upper lobes interstitial involvement, which justifies prompt referral to specialized centers. Given its progressive behavior, negative impact on quality of life, and high mortality, PPFE treatment remains the main challenge for this entity.
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