Kimberly Showalter1, Aileen Hoffmann2, Carrie Richardson3, David Aaby4, Jungwha Lee5, Jane Dematte6, Rishi Agrawal7, Hatice Savas7, Xiaoping Wu8, Rowland W Chang9, Monique Hinchcliff10. 1. K. 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. 2. A. Hoffmann, MS, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Rheumatology, Chicago, Illinois. 3. C. Richardson, MD, Northwestern University Feinberg School of Medicine, Department of Medicine, Chicago, Illinois. 4. D. Aaby, MS, Northwestern University Feinberg School of Medicine, Department of Preventive Medicine, Chicago, Illinois. 5. J. Lee, PhD, MPH, Northwestern University Feinberg School of Medicine, Department of Preventive Medicine, and Institute for Public Health and Medicine, Chicago, Illinois. 6. J. Dematte, MD, MBA, Northwestern University Feinberg School of Medicine, Department of Medicine, Division of Pulmonary and Critical Care Medicine, Chicago, Illinois. 7. R. Agrawal, MD, H. Savas, MD, Northwestern University Feinberg School of Medicine, Department of Radiology, Chicago, Illinois. 8. X. Wu, MD, MS, New York Presbyterian/Weill Cornell, Department of Medicine, Division of Pulmonary and Critical Care Medicine, New York, New York. 9. R.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. 10. M. 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. monique.hinchcliff@yale.edu.
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.
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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