RT Journal Article SR Electronic T1 Characterisation of patients with interstitial pneumonia with autoimmune features JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1767 OP 1775 DO 10.1183/13993003.01565-2015 VO 47 IS 6 A1 Justin M. Oldham A1 Ayodeji Adegunsoye A1 Eleanor Valenzi A1 Cathryn Lee A1 Leah Witt A1 Lena Chen A1 Aliya N. Husain A1 Steven Montner A1 Jonathan H. Chung A1 Vincent Cottin A1 Aryeh Fischer A1 Imre Noth A1 Rekha Vij A1 Mary E. Strek YR 2016 UL http://erj.ersjournals.com/content/47/6/1767.abstract AB Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.IPAF is common among patients with IIP and has distinct subgroups that demonstrate differential survival http://ow.ly/Z0ShD