TY - JOUR
T1 - Esophageal Dilation and Other Clinical Factors Associated with Pulmonary Function Decline in Patients with Systemic Sclerosis
AU - Showalter, Kimberly
AU - Hoffmann, Aileen
AU - Richardson, Carrie
AU - Aaby, David
AU - Lee, Jungwha
AU - Dematte, Jane
AU - Agrawal, Rishi
AU - Savas, Hatice
AU - Wu, Xiaoping
AU - Chang, Rowland W.
AU - Hinchcliff, Monique
N1 - Publisher Copyright:
© 2021 The Journal of Rheumatology
PY - 2021/12/1
Y1 - 2021/12/1
N2 - 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.
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 (≥ 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.
KW - Biomarkers
KW - Gastrointestinal disease
KW - Interstitial lung disease
KW - Systemic sclerosis
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U2 - 10.3899/JRHEUM.210533
DO - 10.3899/JRHEUM.210533
M3 - Article
C2 - 34266985
AN - SCOPUS:85121674923
SN - 0315-162X
VL - 48
SP - 1830
EP - 1838
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 12
ER -