ABSTRACT
Pleuroparenchymal fibroelastosis(PPFE) is a recently characterised type of interstitial lung disease that begins in the upper lung zones and extends to the entire lung. To date, the aetiology is unknown. However, the cause of PPFE in the current case report is rheumatoid arthritis. The symptoms are dyspnoea and dry cough. Pneumothorax is a common complication that occurs at the time of presentation or subsequently in the progression of the disease. It usually occurs in > 40% of ex-smokers with restrictive patterns in the pulmonary function test. Interstitial fibrosis appears pathologically as a thick consolidation insomepreserved alveolar septal outlines and a markedly abrupt contact with thenormal residual lung. Undiagnosed PPFE casescan be misdiagnosed as sarcoidosis, atypical idiopathic pulmonary fibrosis, or other unclassifiable interstitial pneumonia. The current case report will address the rare outline of Rheumatoid arthritis with PPFE.