Related Experiment Video
Updated: Apr 16, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Connective Tissue Disease-associated Interstitial Lung Diseases (CTD-ILD) - Report from OMERACT CTD-ILD Working Group
Dinesh Khanna1, Shikha Mittoo2, Rohit Aggarwal2
1From the Department of Internal Medicine, Division of Rheumatology, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA; University of Toronto, Toronto, Ontario, Canada; University of Pittsburgh, Pittsburgh, Pennsylvania, USA; Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia; University of Auckland, Auckland, New Zealand; Mayo Clinic, Rochester, Minnesota; National Jewish Hospital, Denver, Colorado, USA; Royal Brompton Hospital and National Heart and Lung Institute, London, UK; Scleroderma Research Consultants, Litchfield, Connecticut; Vibeke Strand, MD, Stanford University, Palo Alto, California, USA.D. Khanna, MD, MSc, Associate Professor of Medicine, Director, University of Michigan Scleroderma Program, University of Michigan; S. Mittoo, MD, MHS, University of Toronto; R. Aggarwal, MD, MS, Assistant Professor of Medicine, University of Pittsburgh; S.M. Proudman, MBBS, Royal Adelaide Hospital and Associate Professor Discipline of Medicine, University of Adelaide; N. Dalbeth, MD, FRACP, University of Auckland; E.L. Matteson, MD, Mayo Clinic; K. Brown, MD, National Jewish Hospital; K. Flahery, MD, MSc, Professor of Medicine, University of Michigan; A.U. Wells, MD, Royal Brompton Hospital and National Heart and Lung Institute; J.R. Seibold, MD, Scleroderma Research Consultants; V. Strand, MD, Stanford University. khannad@umich.edu.
Objective:
Interstitial lung disease (ILD) is common in connective tissue disease (CTD) and is the leading cause of mortality. Investigators have used certain outcome measures in randomized controlled trials (RCT) in CTD-ILD, but the lack of a systematically developed, CTD-specific index that captures all measures relevant and meaningful to patients with CTD-ILD has left a large and conspicuous gap in CTD-ILD research.
Methods:
The CTD-ILD working group, under the aegis of the Outcome Measures in Rheumatology (OMERACT) initiative, has completed a consensus group exercise to reach harmony on core domains and items for inclusion in RCT in CTD-ILD. During the OMERACT 12 meeting, consensus was sought on domains and core items for inclusion in RCT. In addition, consensus was pursued on a definition of response in RCT. Consensus was defined as ≥ 75% agreement among the participants.
Results:
OMERACT 12 participants endorsed the domains with minimal modifications. Clinically meaningful progression for CTD-ILD was proposed as ≥ 10% relative decline in forced vital capacity (FVC) or ≥ 5% to < 10% relative decline in FVC and ≥ 15% relative decline in DLCO.
Conclusion:
There is consensus on domains for inclusion in RCT in CTD-ILD and on a definition of clinically meaningful progression. Data-driven approaches to validate these results in different cohorts and RCT are needed.
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