PMID- 27103387 OWN - NLM STAT- MEDLINE DCOM- 20180319 LR - 20220331 IS - 1399-3003 (Electronic) IS - 0903-1936 (Print) IS - 0903-1936 (Linking) VI - 47 IP - 6 DP - 2016 Jun TI - Characterisation of patients with interstitial pneumonia with autoimmune features. PG - 1767-75 LID - 10.1183/13993003.01565-2015 [doi] 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. CI - Copyright (c)ERS 2016. FAU - Oldham, Justin M AU - Oldham JM AD - Dept of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, The University of California at Davis These authors contributed equally joldham@ucdavis.edu. FAU - Adegunsoye, Ayodeji AU - Adegunsoye A AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally. FAU - Valenzi, Eleanor AU - Valenzi E AD - Dept of Medicine, The University of Chicago, Chicago, IL, USA. FAU - Lee, Cathryn AU - Lee C AD - Dept of Medicine, The University of Chicago, Chicago, IL, USA. FAU - Witt, Leah AU - Witt L AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA. FAU - Chen, Lena AU - Chen L AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA. FAU - Husain, Aliya N AU - Husain AN AD - Dept of Pathology, The University of Chicago, Chicago, IL, USA. FAU - Montner, Steven AU - Montner S AD - Dept of Radiology, The University of Chicago, Chicago, IL, USA. FAU - Chung, Jonathan H AU - Chung JH AD - Dept of Radiology, The University of Chicago, Chicago, IL, USA. FAU - Cottin, Vincent AU - Cottin V AD - Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon, Lyon, France. FAU - Fischer, Aryeh AU - Fischer A AD - Dept of Medicine, Division of Rheumatology, University of Colorado, Denver, CO, USA. FAU - Noth, Imre AU - Noth I AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA. FAU - Vij, Rekha AU - Vij R AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally. FAU - Strek, Mary E AU - Strek ME AD - Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA These authors contributed equally. LA - eng GR - T32 HL007605/HL/NHLBI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20160421 PL - England TA - Eur Respir J JT - The European respiratory journal JID - 8803460 RN - 0 (Immunosuppressive Agents) SB - IM CIN - Eur Respir J. 2016 Jun;47(6):1622-4. PMID: 27246077 CIN - Eur Respir J. 2016 Dec;48(6):1811-1813. PMID: 27903692 CIN - Eur Respir J. 2016 Dec;48(6):1813-1814. PMID: 27903693 EIN - Eur Respir J. 2017 May 11;49(5):. PMID: 28495685 MH - Aged MH - Autoimmune Diseases/*diagnosis/*immunology MH - Biopsy MH - Cohort Studies MH - Connective Tissue Diseases/diagnosis/immunology MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/*diagnosis/*immunology MH - Immunosuppressive Agents/therapeutic use MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Retrospective Studies MH - Time Factors MH - Treatment Outcome PMC - PMC5515625 MID - NIHMS877754 COIS- Conflict of interests: Disclosures can be found alongside the online version of this article at erj.ersjournals.com EDAT- 2016/04/23 06:00 MHDA- 2018/03/20 06:00 PMCR- 2017/07/18 CRDT- 2016/04/23 06:00 PHST- 2015/09/20 00:00 [received] PHST- 2016/02/22 00:00 [accepted] PHST- 2016/04/23 06:00 [entrez] PHST- 2016/04/23 06:00 [pubmed] PHST- 2018/03/20 06:00 [medline] PHST- 2017/07/18 00:00 [pmc-release] AID - 13993003.01565-2015 [pii] AID - 10.1183/13993003.01565-2015 [doi] PST - ppublish SO - Eur Respir J. 2016 Jun;47(6):1767-75. doi: 10.1183/13993003.01565-2015. Epub 2016 Apr 21.