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Research ArticleSystemic Sclerosis

Esophageal Dilation and Other Clinical Factors Associated With Pulmonary Function Decline in Patients With Systemic Sclerosis

Kimberly Showalter, Aileen Hoffmann, Carrie Richardson, David Aaby, Jungwha Lee, Jane Dematte, Rishi Agrawal, Hatice Savas, Xiaoping Wu, Rowland W. Chang and Monique Hinchcliff
The Journal of Rheumatology December 2021, 48 (12) 1830-1838; DOI: https://doi.org/10.3899/jrheum.210533
Kimberly Showalter
1K. Showalter, MD, Northwestern University Feinberg School of Medicine, Department of Medicine, Chicago, Illinois, and Hospital for Special Surgery, Department of Medicine, Division of Rheumatology, New York, New York;
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  • ORCID record for Kimberly Showalter
Aileen Hoffmann
2A. Hoffmann, MS, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Rheumatology, Chicago, Illinois;
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Carrie Richardson
3C. Richardson, MD, Northwestern University Feinberg School of Medicine, Department of Medicine, Chicago, Illinois;
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David Aaby
4D. Aaby, MS, Northwestern University Feinberg School of Medicine, Department of Preventive Medicine, Chicago, Illinois;
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Jungwha Lee
5J. Lee, PhD, MPH, Northwestern University Feinberg School of Medicine, Department of Preventive Medicine, and Institute for Public Health and Medicine, Chicago, Illinois;
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Jane Dematte
6J. Dematte, MD, MBA, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Pulmonary and Critical Care Medicine, Chicago, Illinois;
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Rishi Agrawal
7R. Agrawal, MD, H. Savas, MD, Northwestern University Feinberg School of Medicine, Department of Radiology, Chicago, Illinois;
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Hatice Savas
7R. Agrawal, MD, H. Savas, MD, Northwestern University Feinberg School of Medicine, Department of Radiology, Chicago, Illinois;
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Xiaoping Wu
8X. Wu, MD, MS, New York Presbyterian/Weill Cornell, Department of Medicine, Division of Pulmonary and Critical Care Medicine, New York, New York;
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Rowland W. Chang
9R.W. Chang, MD, MPH, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Rheumatology, Department of Preventive Medicine, and Institute for Public Health and Medicine, Chicago, Illinois;
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Monique Hinchcliff
10M. Hinchcliff, MD, MS, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Rheumatology, Chicago, Illinois, and Yale School of Medicine, Department of Medicine, Section of Rheumatology, Allergy & Immunology, New Haven, Connecticut, USA.
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  • For correspondence: monique.hinchcliff@yale.edu
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Abstract

Objective To identify clinical factors, including esophageal dilation on chest high-resolution computed tomography (HRCT), that are associated with pulmonary function decline in patients with systemic sclerosis (SSc).

Methods Patients fulfilled 2013 SSc criteria and had ≥ 1 HRCT and ≥ 2 pulmonary function tests (PFTs). According to published methods, widest esophageal diameter (WED) and radiographic interstitial lung disease (ILD) were assessed, and WED was dichotomized as dilated (≥ 19 mm) vs not dilated (< 19 mm). Clinically meaningful PFT decline was defined as % predicted change in forced vital capacity (FVC) ≥ 5 and/or diffusion capacity for carbon monoxide (DLCO) ≥ 15. Linear mixed effects models were used to model PFT change over time.

Results One hundred thirty-eight patients with SSc met the study criteria: 100 (72%) had radiographic ILD; 49 (35%) demonstrated FVC decline (median follow-up 2.9 yrs). Patients with antitopoisomerase I (Scl-70) autoantibodies had 5-year FVC% predicted decline (–6.33, 95% CI –9.87 to –2.79), whereas patients without Scl-70 demonstrated 5-year FVC stability (+1.78, 95% CI –0.59 to 4.15). Esophageal diameter did not distinguish between those with vs without FVC decline. Patients with esophageal dilation had statistically significant 5-year DLCO% predicted decline (–5.58, 95% CI –10.00 to –1.15), but this decline was unlikely clinically significant. Similar results were observed in the subanalysis of patients with radiographic ILD.

Conclusion In patients with SSc, Scl-70 positivity is a risk factor for FVC% predicted decline at 5 years. Esophageal dilation on HRCT was associated with a minimal, nonclinically significant decline in DLCO and no change in FVC during the 5-year follow-up. These results have prognostic implications for SSc-ILD patients with esophageal dilation.

Key Indexing Terms:
  • biomarkers
  • gastrointestinal disease
  • interstitial lung disease
  • systemic sclerosis
  • Accepted for publication July 8, 2021.
  • Copyright © 2021 by the Journal of Rheumatology
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The Journal of Rheumatology
Vol. 48, Issue 12
1 Dec 2021
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Esophageal Dilation and Other Clinical Factors Associated With Pulmonary Function Decline in Patients With Systemic Sclerosis
Kimberly Showalter, Aileen Hoffmann, Carrie Richardson, David Aaby, Jungwha Lee, Jane Dematte, Rishi Agrawal, Hatice Savas, Xiaoping Wu, Rowland W. Chang, Monique Hinchcliff
The Journal of Rheumatology Dec 2021, 48 (12) 1830-1838; DOI: 10.3899/jrheum.210533

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Esophageal Dilation and Other Clinical Factors Associated With Pulmonary Function Decline in Patients With Systemic Sclerosis
Kimberly Showalter, Aileen Hoffmann, Carrie Richardson, David Aaby, Jungwha Lee, Jane Dematte, Rishi Agrawal, Hatice Savas, Xiaoping Wu, Rowland W. Chang, Monique Hinchcliff
The Journal of Rheumatology Dec 2021, 48 (12) 1830-1838; DOI: 10.3899/jrheum.210533
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Keywords

BIOMARKERS
gastrointestinal disease
INTERSTITIAL LUNG DISEASE
SYSTEMIC SCLEROSIS

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