PMID- 31387157 OWN - NLM STAT- MEDLINE DCOM- 20210622 LR - 20210622 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 25 IP - 6 DP - 2020 Jun TI - Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. PG - 636-643 LID - 10.1111/resp.13665 [doi] AB - BACKGROUND AND OBJECTIVE: Idiopathic interstitial pneumonia (IIP) with autoimmune features that does not fulfil connective tissue disease (CTD) criteria has been recently defined as interstitial pneumonia with autoimmune features (IPAF). However, its long-term clinical course and outcome are poorly understood. METHODS: We included consecutive patients diagnosed with IIP (n = 586) or CTD-related interstitial lung disease (CTD-ILD, n = 149). Some patients with IIP were reclassified as IPAF based on recent guidelines. RESULTS: The median follow-up period was 45 months. Among the IIP patients, 109 (18.6%) were reclassified as IPAF. Compared to the non-IPAF-IIP group, the IPAF group had slower diffusing capacity of the lung for carbon monoxide (DL(CO) ) and total lung capacity declines, and more frequent CTD development during follow-up periods. The prognosis of the IPAF was better than that of the non-IPAF-IIP and similar to that of the CTD-ILD. IPAF was associated with better prognosis in the IIP cohort on univariate but not on multivariate analysis. Usual interstitial pneumonia (UIP) pattern, old age and low DL(CO) independently predicted mortality in the IPAF group. CONCLUSION: Compared to the non-IPAF-IIP group, the IPAF group had slower lung function declines and more frequent CTD development during follow-up. Although the prognosis of IPAF group was better than that of non-IPAF-IIP group and similar to that of CTD-ILD group, it showed poor prognosis in patients with old age, UIP pattern, and low DL(CO) . CI - (c) 2019 Asian Pacific Society of Respirology. FAU - Kim, Ho Cheol AU - Kim HC AD - Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. FAU - Lee, Joo Hee AU - Lee JH AUID- ORCID: 0000-0002-5923-0586 AD - Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. FAU - Chae, Eun Jin AU - Chae EJ AD - Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. FAU - Song, Joon Seon AU - Song JS AD - Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. FAU - Song, Jin Woo AU - Song JW AUID- ORCID: 0000-0001-5121-3522 AD - Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. LA - eng GR - NRF-2016R1A2B4016318/National Research Foundation of Korea/ PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190806 PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 SB - IM MH - Aged MH - Cohort Studies MH - Connective Tissue Diseases/*physiopathology MH - Female MH - Humans MH - Idiopathic Interstitial Pneumonias/*physiopathology/*therapy MH - Lung/*physiopathology MH - Male MH - Middle Aged MH - Multivariate Analysis MH - Prognosis MH - Respiratory Function Tests OTO - NOTNLM OT - connective tissue disease-associated interstitial lung disease OT - idiopathic interstitial pneumonia OT - interstitial pneumonia with autoimmune features OT - prognosis EDAT- 2019/08/07 06:00 MHDA- 2021/06/23 06:00 CRDT- 2019/08/07 06:00 PHST- 2019/02/06 00:00 [received] PHST- 2019/06/11 00:00 [revised] PHST- 2019/07/15 00:00 [accepted] PHST- 2019/08/07 06:00 [pubmed] PHST- 2021/06/23 06:00 [medline] PHST- 2019/08/07 06:00 [entrez] AID - 10.1111/resp.13665 [doi] PST - ppublish SO - Respirology. 2020 Jun;25(6):636-643. doi: 10.1111/resp.13665. Epub 2019 Aug 6.