Rapidly Progressive Pleuro-parenchymal Fibroelastosis Secondary to Cyclophosphamide Chemotherapy

Bhaskar, Maheema; Tisekar, Owais; Tandon, Sandeep; Vora, Tushar; Jankharia, Bhavin

doi:10.4103/ijmpo.ijmpo_85_19

Cited by 2 publications

(1 citation statement)

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“…Pleuroparenchymal fibroelastosis (PPFE) is an under and misdiagnosed clinicopathological entity characterized by fibroelastosis of the pleura and subpleural lung parenchyma with striking upper lobe predominance. Multiple sporadic cases and case series have been reported from all over the globe [1][2][3][4][5][6]. To the best of our knowledge, this is the third case of idiopathic pleuroparenchymal fibroelastosis (IPPFE) to be reported from our subcontinent and the second case for which the diagnosis was made antemortem.…”

Section: Introductionmentioning

confidence: 77%

Overcoming the challenges of a misdiagnosed rare lung disease - Idiopathic pleuroparenchymal fibroelastosis

Garg

Upadhya

Kumar

et al. 2023

Monaldi Arch Chest Dis

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Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by pleural and subpleural lung fibroelastosis with an upper lobe predominance. We present the third case of idiopathic pleuroparenchymal fibroelastosis (IPPFE) from India, as well as the second antemortem diagnosis. A 27-year-old man presented with a one-year history of mMRC class II dry cough and shortness of breath. He described a fifteen-kilogram weight loss. After a clinico-radiological diagnosis, he was given anti-tubercular treatment and referred because he showed no improvement. A high-resolution computed tomography of the chest revealed bilateral upper lobe bullae, parenchymal and subpleural fibrosis, and irregular pleural thickening. PPFE was found in surgical lung and pleural biopsies. He was given systemic glucocorticoids but did not respond clinically or radiologically. Pirfenidone and a lung transplant were out of reach for him. He died nine months after being diagnosed with his condition. Finally, IPPFE is an extremely rare entity, with only three cases reported from our subcontinent. As a result, it is easily underdiagnosed or misdiagnosed; clinician awareness of this condition is critical for better diagnosis and management.

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