PMID- 28300570 OWN - NLM STAT- MEDLINE DCOM- 20170831 LR - 20181115 IS - 1931-3543 (Electronic) IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 152 IP - 1 DP - 2017 Jul TI - Idiopathic Interstitial Pneumonia Associated With Autoantibodies: A Large Case Series Followed Over 1 Year. PG - 103-112 LID - S0012-3692(17)30364-1 [pii] LID - 10.1016/j.chest.2017.03.004 [doi] AB - BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia, particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease-associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF), or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: This was a retrospective single-center study. Analyses of variance compared differences in mean change in FVC and diffusion capacity (Dlco) over 1 year among 124 well-defined patients (20 patients with positive autoantibodies with or without symptoms of connective tissue disease [AI-ILD], 15 patients with IPAF, 36 patients with CTD-ILD, and 53 patients with IPF with negative CTD serologies [Lone-IPF]). RESULTS: Of the patients, 75% with AI-ILD, 33% with IPAF, and 33% with CTD-ILD had UIP. Initial FVC and Dlco were similarly moderately reduced across groups. Mean change in FVC over 12 months was as follows: -60 mL (IPAF), -110 mL (AI-ILD), -10 mL (CTD-ILD), and -90 mL (Lone-IPF) (P = .52). Mean change in Dlco was as follows: 2.39 mL/mm Hg/min (IPAF), -1.15 mL/mm Hg/min (AI-ILD), -0.27 mL/mm Hg/min (CTD-ILD), and -1.05 mL/mm Hg/min (Lone-IPF) (P < .001). By pattern of disease, the mean change in FVC was as follows: -140 mL (UIP), 10 mL (nonspecific interstitial pneumonia), and 12 mL (unclassifiable/other) (P = .001). CONCLUSIONS: No clinically significant differences in pulmonary function to distinguish between patients with AI-ILD, IPAF, CTD-ILD, and Lone-IPF were observed after 1 year. Longer periods of follow-up are needed to understand the outcomes of these patients. It is not yet clear whether AI-ILD is a distinct phenotype or a variant of the newly proposed entity IPAF. CI - Copyright (c) 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved. FAU - Collins, Bridget F AU - Collins BF AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Spiekerman, Charles F AU - Spiekerman CF AD - Institute of Translational Health Sciences, University of Washington Medical Center, Seattle, WA. FAU - Shaw, Megan A AU - Shaw MA AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Ho, Lawrence A AU - Ho LA AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Hayes, Jennifer AU - Hayes J AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Spada, Carolyn A AU - Spada CA AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Stamato, Caroline M AU - Stamato CM AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. FAU - Raghu, Ganesh AU - Raghu G AD - Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA. Electronic address: graghu@uw.edu. LA - eng GR - UL1 TR000423/TR/NCATS NIH HHS/United States GR - UL1 TR002319/TR/NCATS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20170312 PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Autoantibodies/*blood MH - Autoimmunity/immunology MH - Cohort Studies MH - *Connective Tissue Diseases/diagnosis/physiopathology MH - Diagnosis, Differential MH - Female MH - Follow-Up Studies MH - Humans MH - *Idiopathic Interstitial Pneumonias/diagnosis/epidemiology/immunology/physiopathology MH - *Idiopathic Pulmonary Fibrosis/diagnosis/immunology/physiopathology MH - Lung/diagnostic imaging/physiopathology MH - *Lung Diseases, Interstitial/diagnosis/immunology/physiopathology MH - Male MH - Middle Aged MH - Needs Assessment MH - Respiratory Function Tests/methods MH - Statistics as Topic MH - Tomography, X-Ray Computed/methods MH - Washington/epidemiology PMC - PMC6026269 OTO - NOTNLM OT - autoimmune interstitial lung disease OT - idiopathic pulmonary fibrosis OT - interstitial lung disease OT - interstitial pneumonia with autoimmune features EDAT- 2017/03/17 06:00 MHDA- 2017/09/01 06:00 PMCR- 2018/07/01 CRDT- 2017/03/17 06:00 PHST- 2016/09/23 00:00 [received] PHST- 2017/02/01 00:00 [revised] PHST- 2017/03/01 00:00 [accepted] PHST- 2017/03/17 06:00 [pubmed] PHST- 2017/09/01 06:00 [medline] PHST- 2017/03/17 06:00 [entrez] PHST- 2018/07/01 00:00 [pmc-release] AID - S0012-3692(17)30364-1 [pii] AID - 10.1016/j.chest.2017.03.004 [doi] PST - ppublish SO - Chest. 2017 Jul;152(1):103-112. doi: 10.1016/j.chest.2017.03.004. Epub 2017 Mar 12.