Interstitial Pneumonia with Autoimmune Features: Challenges and Controversies

The document discusses the challenges and controversies surrounding Interstitial Pneumonia with Autoimmune Features (IPAF), a condition that falls between idiopathic interstitial pneumonia and connective tissue disease-associated interstitial lung disease. The authors emphasize the need for a multidisciplinary approach to accurately diagnose and manage IPAF, and the need for further research to establish more precise diagnostic criteria and effective treatment strategies. The paper also highlights a case of an 84-year-old female patient with IPAF and the difficulties in identifying co-existent disease processes due to the high prevalence of comorbidities in patients with Interstitial Lung Disease (ILD). The document concludes by emphasizing the need for continual evaluation for the presence of Connective Tissue Disease symptoms in all patients with ILD, including IPAF. Treatment strategies for IPAF are largely extrapolated from Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD) studies and are based on expert opinion. A retrospective cohort of 52 patients showed potential benefits from mycophenolate therapy, and a phase 2 trial with 253 patients suggested benefits from pirfenidone and nintedanib treatment. However, treatment decisions must be based on a careful evaluation of benefit to risk ratio in the individual subject.