Research ArticleArticle

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 July 2021, jrheum.210533; DOI: https://doi.org/10.3899/jrheum.210533

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.

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