PMID- 29320807 OWN - NLM STAT- MEDLINE DCOM- 20181114 LR - 20181114 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 23 IP - 6 DP - 2018 Jun TI - Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality. PG - 600-605 LID - 10.1111/resp.13254 [doi] AB - BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome. METHODS: Patients with prior UCTD-interstitial lung disease (ILD) were screened by ERS/ATS criteria for IPAF. Clinical data along with all-cause mortality were collated and compared with selected idiopathic pulmonary fibrosis (IPF) patients from the same study period. Survival was compared between IPAF subgroups with and without UIP features. RESULTS: One hundred and one UCTD-ILD subjects (91%) evaluated from 2005 to 2012 also met strict criteria for IPAF. Frequent clinical findings included Raynaud's phenomenon, positive anti-nuclear antibody (ANA) and non-specific interstitial pneumonia (NSIP) pattern on chest computed tomography (CT). Nineteen had features of UIP either on histopathology or CT imaging. As compared with IPF, IPAF patients had overall better survival except in those with UIP features. CONCLUSION: Current IPAF criteria encompassed the majority of broadly defined UCTD-ILD and included those with UIP findings. Survival compared with IPF in those with UIP was similar. Further studies are necessary to refine IPAF definitions for clinical use and guide directed management strategies. CI - (c) 2018 Asian Pacific Society of Respirology. FAU - Kelly, Bryan T AU - Kelly BT AD - Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA. FAU - Moua, Teng AU - Moua T AUID- ORCID: 0000-0003-3329-5717 AD - Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. LA - eng PT - Journal Article DEP - 20180110 PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 SB - IM CIN - Respirology. 2018 Oct;23(10):959. PMID: 30070408 CIN - Respirology. 2018 Oct;23(10):958. PMID: 30070755 MH - Adult MH - Aged MH - Aged, 80 and over MH - Autoimmune Diseases/*classification/diagnostic imaging/mortality/pathology MH - Cause of Death MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/*classification/diagnostic imaging/mortality/pathology MH - Lung/diagnostic imaging/pathology MH - Lung Diseases, Interstitial/*classification/diagnostic imaging/mortality/pathology MH - Male MH - Middle Aged MH - Mortality MH - Tomography, X-Ray Computed/methods MH - Undifferentiated Connective Tissue Diseases/*classification/diagnostic imaging/mortality/pathology MH - Young Adult OTO - NOTNLM OT - autoimmune disease OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - interstitial pneumonia OT - usual interstitial pneumonia EDAT- 2018/01/11 06:00 MHDA- 2018/11/15 06:00 CRDT- 2018/01/11 06:00 PHST- 2017/02/17 00:00 [received] PHST- 2017/11/22 00:00 [revised] PHST- 2017/12/13 00:00 [accepted] PHST- 2018/01/11 06:00 [pubmed] PHST- 2018/11/15 06:00 [medline] PHST- 2018/01/11 06:00 [entrez] AID - 10.1111/resp.13254 [doi] PST - ppublish SO - Respirology. 2018 Jun;23(6):600-605. doi: 10.1111/resp.13254. Epub 2018 Jan 10.