Title

Baseline characteristics of systemic sclerosis patients with restrictive lung disease in a multi-center US-based longitudinal registry

Authors

Flavia V. Castelino, Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
John M. VanBuren, Department or Pediatrics, University of Utah, Salt Lake City, Utah, USA.
Emily Startup, Department or Pediatrics, University of Utah, Salt Lake City, Utah, USA.
Shervin Assassi, The University of Texas Health Science Center at Houston, Houston, Texas, USA.
Elana J. Bernstein, Division of Rheumatology, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, New York, USA.
Lorinda Chung, Division of Rheumatology, Department of Medicine and Dermatology, Stanford University and Palo Alto Veterans Affairs Health Care System, Stanford, California, USA.
Chase Correia, Division of Rheumatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Luke B. Evnin, Scleroderma Research Foundation, San Francisco, California, USA.
Tracy M. Frech, Division of Rheumatology, Department of Internal Medicine, University of Utah and Salt Lake Veterans Affair Medical Center, Salt Lake City, Utah, USA.
Jessica K. Gordon, Hospital for Special Surgery, New York, New York, USA.
Faye N. Hant, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, South Carolina, USA.
Laura K. Hummers, Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Dinesh Khanna, University of Michigan Scleroderma Program, Ann Arbor, Michigan, USA.
Nora Sandorfi, Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Ami A. Shah, Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Victoria K. Shanmugam, Division of Rheumatology, The George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Virginia Steen, Georgetown University School of Medicine, Washington, District of Columbia, USA.

Document Type

Journal Article

Publication Date

2-1-2022

Journal

International journal of rheumatic diseases

Volume

25

Issue

2

DOI

10.1111/1756-185X.14253

Keywords

interstitial lung disease; registry; restrictive lung disease; scleroderma; systemic sclerosis

Abstract

AIM: Interstitial lung disease (ILD) is the leading cause of disease-related death in systemic sclerosis (SSc). Here, we assess baseline characteristics of SSc subjects with and without restrictive lung disease (RLD) in a multi-center, US-based registry. METHODS: SSc patients within 5 years of disease onset were enrolled in the Collaborative National Quality and Efficacy Registry (CONQUER), a multi-center US-based registry of SSc study participants (age ≥ 18 years) enrolled at 13 expert centers. All subjects met 2013 American College of Rheumatology / European League Against Rheumatism criteria. Subjects with a pulmonary function test (PFT) at baseline before April 1, 2020 were included. High-resolution computed tomography scan of the chest was not available to characterize ILD for all subjects. RLD was defined as forced vital capacity (FVC) <80% or total lung capacity (TLC) <80% predicted. RESULTS: There were 160 (45%) SSc subjects characterized as having RLD. There was no significant difference in age, gender or disease duration. RLD subjects had a mean disease duration from date of first non-Raynaud's symptom of 2.6 years and a mean FVC% predicted of 67% at baseline. In multivariable analysis, non-White race, higher physician global health assessment and modified Medical Research Council (mMRC) dyspnea scores, were independently associated with RLD. In the subgroup of RLD subjects with ILD, ILD had a negative correlation with RNA polymerase III antibody. CONCLUSION: CONQUER is the largest, multi-center, prospective cohort of early SSc patients in the US. Non-White race was independently associated with RLD. In addition, 45% of CONQUER subjects already had RLD, highlighting the importance of screening for SSc-ILD at initial diagnosis.

Department

Anatomy and Regenerative Biology

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