https://doi.org/10.4081/monaldi.2023.2632
Overcoming the challenges of a misdiagnosed rare lung disease – idiopathic pleuroparenchymal fibroelastosis
Submitted: May 8, 2023
Accepted: June 26, 2023
Published: August 2, 2023
Accepted: June 26, 2023
Abstract Views: 1209
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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 PPFE from India, as well as the second ante-mortem diagnosis. A 27-year-old man presented with a 1-year history of modified Medical Research Council class II shortness of breath and dry cough. He described a 15-kg 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 9 months after being diagnosed with his condition. Finally, idiopathic PPFE 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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How to Cite
Garg, Shivam, Pratap Upadhya, Arul Kumar, Norton Stephen, and Bala Subramanian. 2023. “Overcoming the Challenges of a Misdiagnosed Rare Lung Disease – Idiopathic Pleuroparenchymal Fibroelastosis”. Monaldi Archives for Chest Disease 94 (2). https://doi.org/10.4081/monaldi.2023.2632.
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