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Original Research |

Idiopathic Interstitial Pneumonia associated with autoantibodies: A large case series followed over one year

Bridget F. Collins, MD; Charles F. Spiekerman, PhD; Megan A. Shaw, MD; Lawrence A. Ho, MD; Jennifer Hayes, RN; Carolyn A. Spada, RN; Caroline M. Stamato, MPH; Ganesh Raghu, MD
Author and Funding Information

Author Contributions:

Dr. Collins contributed to the design of the study, data acquisition, interpretation of data, drafting and revising the manuscript, and provided final approval of the manuscript.

Dr Shaw contributed to design of the study, data acquisition, revising the manuscript, and final approval of the manuscript.

Dr Ho, Jennifer Hayes, Caroline Stamato, and Carolyn Spada contributed to data acquisition, revising the manuscript, and final approval of the manuscript.

Dr. Spiekerman contributed to the design of the study, analysis of the data, revision of the manuscript, and provided final approval of the manuscript

Dr. Raghu initiated, planned and contributed to the design of the study, interpretation of data, drafting and revising the manuscript, supervised the study and provided final approval of the manuscript.

Responsibility for data: Drs. Collins and Raghu had full access to all of the data and take full responsibility for the integrity of the data and the accuracy of the data analyses.

Sources of support: This project was in part supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under Award Number UL1TR000423. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health

Declaration of Interests: JH has participated on an advisory board and as a healthcare consultant for Boehringer Ingelheim. None declared for all others.

1Center for Interstitial Lung Diseases (CILD), University of Washington Medical Center, Seattle WA

2Institute of Translational Health Sciences, University of Washington Medical Center, Seattle WA

Corresponding author: Ganesh Raghu University of Washington Medical Center Center for Interstitial Lung Diseases 1959 NE Pacific St Campus Box 356175 Seattle, WA 98195.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.03.004
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Abstract

Background  Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), 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  Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 “Lone-IPF” [patients with IPF without any autoantibodies])

Results  75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP. Initial FVC and DLCO were similarly moderately reduced across groups. Mean change in FVC: -60 mL IPAF, -110 mL AI-ILD, -10 mL CTD-ILD, -90 mL Lone-IPF (p=0.52); Mean change in DLCO (mL/mmHg/min): 2.39 IPAF, -1.15 AI-ILD, -0.27 CTD-ILD, -1.05 Lone-IPF (p < 0.001) over 12 months. By pattern of disease mean change in FVC: -140 mL UIP, 10 mL NSIP, 12 mL unclassifiable/other (p=0.001).

Conclusion  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.


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