An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

TLDR

Many patients with idiopathic interstitial pneumonia exhibit autoimmune features yet do not meet established connective tissue disease criteria, and the absence of consensus on terminology hampers prospective studies. The European Respiratory Society/American Thoracic Society Task Force was convened to establish consensus on nomenclature and classification for these patients. They introduced the term interstitial pneumonia with autoimmune features (IPAF) and defined criteria based on clinical, serologic, and morphologic domains. IPAF offers a standardized framework that enables future research on a more uniform cohort.

Abstract

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features 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, 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 features 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 features 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. A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.