The research progress of interstitial pneumonia with autoimmune features

Abstract

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, such as undifferentiated connective tissue disease (UCTD), lung-dominant connective tissue disease (Lung-dominant CTD), autoimmune-featured ILD, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and of autoimmunity.The task force proposes the term interstitial pneumonia with autoimmune features (IPAF) and offers classification criteria organised around the presence of a combination of from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort. Key words: Interstitial pneumonia with autoimmune features; Undifferentiated connective tissue disease; Lung-dominant connective tissue disease; Autoimmune-featured ILD