PMID- 31831537 OWN - NLM STAT- MEDLINE DCOM- 20201104 LR - 20201104 IS - 2044-6055 (Electronic) IS - 2044-6055 (Linking) VI - 9 IP - 12 DP - 2019 Dec 11 TI - Systematic review and meta-analysis of the prognosis and prognostic factors of interstitial pneumonia with autoimmune features. PG - e031444 LID - 10.1136/bmjopen-2019-031444 [doi] LID - e031444 AB - OBJECTIVE: To clarify the prognosis and prognostic factors of interstitial pneumonia with autoimmune features (IPAF) in comparison to idiopathic pulmonary fibrosis (IPF), the most common idiopathic interstitial pneumonia, and connective tissue disease-associated interstitial pneumonia (CTD-IP). DESIGN: A systematic review and meta-analysis. DATA SOURCES: Electronic databases such as Medline and Embase were searched from 2015 through 6 September 2019. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: Primary studies that comparatively investigated the prognosis or prognostic factors of IPAF were eligible. DATA EXTRACTION AND ANALYSIS: Two reviewers extracted relevant data and assessed the risk of bias independently. A meta-analysis was conducted using a random-effects model. The quality of presented evidence was assessed by the Grades of Recommendation, Assessment, Development, and Evaluation system. RESULTS: Out of a total of 656 records retrieved, 12 studies were reviewed. The clinical features of IPAF were diverse between studies, which included a radiological and/or pathological usual interstitial pneumonia (UIP) pattern of between 0% and 73.8%. All studies contained some risk of bias. There was no significant difference of all-cause mortality between IPAF-UIP and IPF in all studies, although the prognosis of IPAF in contrast to IPF or CTD-IP varied between studies depending on the proportion of UIP pattern. Among the potential prognostic factors identified, age was significantly associated with all-cause mortality of IPAF by a pooled analysis of univariate results with a hazard ratio (HR) of 1.06 (95% confidence interval (CI) 1.04 to 1.07). The adjusted effect of age was also significant in all studies. The quality of presented evidence was deemed as very low. CONCLUSION: There was no significant difference of all-cause mortality between IPAF-UIP and IPF. Age was deemed as a prognostic factor for all-cause mortality of IPAF. The findings should be interpreted cautiously due to the low quality of the presented evidence. PROSPERO REGISTRATION NUMBER: CRD42018115870. CI - (c) Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. FAU - Kamiya, Hiroyuki AU - Kamiya H AUID- ORCID: 0000-0001-5623-1279 AD - School of Population and Global Health, University of Western Australia, Crawley, Western Australia, Australia mlb04194@nifty.com. FAU - Panlaqui, Ogee Mer AU - Panlaqui OM AD - Department of Intensive Care Medicine, Northern Hospital, Epping, Victoria, Australia. LA - eng PT - Journal Article PT - Meta-Analysis PT - Systematic Review DEP - 20191211 PL - England TA - BMJ Open JT - BMJ open JID - 101552874 SB - IM MH - Autoimmune Diseases/diagnostic imaging/*mortality/pathology MH - Cause of Death MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnostic imaging/mortality/pathology MH - Lung/diagnostic imaging/pathology MH - Lung Diseases, Interstitial/diagnostic imaging/*mortality/pathology MH - Prognosis MH - Tomography, X-Ray Computed MH - Undifferentiated Connective Tissue Diseases/diagnostic imaging/mortality/pathology PMC - PMC6924795 OTO - NOTNLM OT - Interstitial pneumonia with autoimmune features OT - meta-analysis OT - prognosis OT - review COIS- Competing interests: None declared. EDAT- 2019/12/14 06:00 MHDA- 2020/11/05 06:00 PMCR- 2019/12/11 CRDT- 2019/12/14 06:00 PHST- 2019/12/14 06:00 [entrez] PHST- 2019/12/14 06:00 [pubmed] PHST- 2020/11/05 06:00 [medline] PHST- 2019/12/11 00:00 [pmc-release] AID - bmjopen-2019-031444 [pii] AID - 10.1136/bmjopen-2019-031444 [doi] PST - epublish SO - BMJ Open. 2019 Dec 11;9(12):e031444. doi: 10.1136/bmjopen-2019-031444.