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

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.