Pleuroparenchymal fibroelastosis in a patient with relapsing polychondritis and ulcerative colitis

Abstract

A woman in her 50s with relapsing polychondritis and ulcerative colitis presented with progressive dyspnoea and dry cough. Physical examination revealed platythorax and coarse crackles in the upper lung zones. High-resolution CT showed bilateral apical pleural thickening and upper lobe fibrosis, while pulmonary function tests indicated a restrictive pattern. Histopathology confirmed pleuroparenchymal fibroelastosis (PPFE) based on a wedge resection and surgical pleurodesis performed due to spontaneous pneumothorax. The patient was treated with prednisolone, which initially stabilised her condition; however, the disease later progressed, leading to respiratory failure. This case highlights the potential association between PPFE and ulcerative colitis, emphasising the need for heightened awareness of atypical pulmonary manifestations in patients with inflammatory bowel disease. Early recognition and a multidisciplinary approach are essential for effective diagnosis and management of this rare interstitial lung disease.