TY - JOUR T1 - Interstitial pneumonitis with autoimmune features (IPAF): a work in progress JF - European Respiratory Journal JO - Eur Respir J SP - 1622 LP - 1624 DO - 10.1183/13993003.00690-2016 VL - 47 IS - 6 AU - Fabrizio Luppi AU - Athol U. Wells Y1 - 2016/06/01 UR - http://erj.ersjournals.com/content/47/6/1622.abstract N2 - In clinical practice, it is common to encounter patients with an “idiopathic” interstitial pneumonia (IIP) associated with features suggestive of, but not diagnostic for, a classical connective tissue disease (CTD). In some cases, serological abnormalities are absent and in other cases, serological findings are suggestive of a specific CTD, but systemic clinical features are not. Alternatively, thoracic radiological or histopathological features are compatible with or highly suggestive of an underlying CTD, but diagnostic criteria built on serological and systemic clinical findings are not met. In suspected idiopathic pulmonary fibrosis (IPF), in particular, the designation of an underlying autoimmune process has profound management implications. The medical literature is punctuated by statements and series exploring aspects of this demi-semi-syndrome, variously termed “undifferentiated CTD associated interstitial lung disease” (UCTD-ILD), “lung-dominant CTD” and “autoimmune-featured ILD” [1–4]. However, the absence of standardised criteria has hindered progress and a new beginning was necessary. In a recent European Respiratory Society/American Thoracic Society research statement [5], the term “interstitial pneumonia with autoimmune features” (IPAF) was proposed to describe the presence of a combination of features from any two of three domains: a clinical domain, consisting of specific extrathoracic features; a serological domain, consisting of specific autoantibodies; and a morphological domain, consisting of specific chest imaging, histopathological or pulmonary physiological features.For the IPAF definition to be accepted, it needs to be shown to add information on the likely future disease course http://ow.ly/10zy1P ER -