ABSTRACT Interstitial Pneumonia with Autoimmune Features (IPAF) refers to a subgroup of interstitial lung diseases that show autoimmune‐like characteristics but do not align with established conditions such as idiopathic pulmonary fibrosis (IPF) or recognized connective tissue diseases (CTD). In this case report, we present a 43‐year‐old woman who had a history of dry cough, decreased appetite, weight loss, and progressive shortness of breath. Initial investigations, including spirometry, autoantibody testing, and CT scan, revealed evidence of nonspecific interstitial pneumonia and positive autoantibodies. Our patient did not fully meet the CTD criteria, and based on the clinical symptoms, imaging findings, and the presence of autoantibodies, an IPAF diagnosis was made. Our patient was treated with prednisolone and mycophenolate, significantly improving her symptoms, pulmonary function, and exercise capacity during the one‐year follow‐up period. Our report mentioned the importance of considering IPAF in the differential diagnosis of patients presenting with interstitial lung disease and features of an autoimmune process. Delayed diagnosis contributes to poorer prognoses and increased disease severity among affected individuals.
Jalayeri et al. (Fri,) studied this question.