RT Journal Article
SR Electronic
T1 Esophageal Dilation and Other Clinical Factors Associated with Pulmonary Function Decline in Patients with Systemic Sclerosis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.210533
DO 10.3899/jrheum.210533
A1 Kimberly Showalter
A1 Aileen Hoffmann
A1 Carrie Richardson
A1 David Aaby
A1 Jungwha Lee
A1 Jane Dematte
A1 Rishi Agrawal
A1 Hatice Savas
A1 Xiaoping Wu
A1 Rowland W.  Chang
A1 Monique Hinchcliff
YR 2021
UL http://www.jrheum.org/content/early/2021/07/11/jrheum.210533.abstract
AB 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 (≥19mm) vs. not dilated (<19mm). 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 effect models were used to model PFT change over time. Results 138 SSc patients met study criteria: 100 (72%) had radiographic ILD; 49 (35%) demonstrated FVC decline (median follow-up 2.9y). Patients with Scl-70 autoantibodies had 5- year %-predicted FVC decline (-6.3; 95% CI -9.9, -2.8), while patients without Scl-70 autoantibodies demonstrated 5-year FVC stability (+1.78; 95% CI -0.6, 4.15). Esophageal diameter did not distinguish between those with vs. without FVC decline. Patients with esophageal dilation had statistically significant 5-year %-predicted DLCO decline (-5.6; 95% CI - 10.0, -1.2), but this decline was unlikely clinically significant. Similar results were observed in sub-analysis of patients with radiographic ILD. Conclusion In patients with SSc, Scl-70 positivity is a risk factor for %-predicted FVC decline at five years. Esophageal dilation on HRCT was associated with a minimal, non-clinically significant decline in DLCO and no change in FVC during 5-year follow-up. These results have prognostic implications for SSc-ILD patients with esophageal dilation.