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Abstract Number: 805

Diastolic Dysfunction in Scleroderma: An Investigation into Associated Risk Factors and Impact on Survival

Alicia M. Hinze1,2, Laura K. Hummers1, Fredrick M. Wigley1, Monica Mukherjee3 and Ami A. Shah1, 1Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 2Division of Rheumatology, Mayo Clinic, Rochester, MN, 3Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: autoantibodies, Cardiovascular disease, morbidity and mortality, risk assessment and scleroderma

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Session Information

Date: Sunday, October 21, 2018

Session Title: Systemic Sclerosis and Related Disorders – Clinical Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Scleroderma heart disease often goes unrecognized until severe clinical manifestations are present. Diastolic dysfunction (DD) may identify patients at risk for cardiac complications by identifying patients with myocardial fibrosis.  The aims of this study were to 1) determine the prevalence of non-valvular DD in scleroderma, 2) assess risk factors for DD in scleroderma, and 3) evaluate the impact of DD on survival.

Methods: A total of 754 subjects with scleroderma seen between November 1st, 2007 through October 31st, 2017 were included in this retrospective cohort study. Echocardiograms were excluded if there was moderate to severe mitral or aortic valve disease, a primary cardiomyopathy, a recent myocardial infarction, or if subjects were admitted to an intensive care unit. The most recent analyzable echocardiogram for each subject was included. DD was defined according to the 2016 American Society of Echocardiography (ASE)/European Society of Cardiovascular Imaging (EACVI) guidelines. Univariable logistic regression assessed risk factors for DD, and a multivariable regression model was constructed of covariates with a p-value of ²0.1.  Inclusion of all traditional DD risk factors in the multivariable model was planned a priori. A Kaplan-Meier curve evaluated for differences in survival among those with and without DD, and a log-rank test for equality of survivor functions evaluated for significance.

Results: The prevalence of DD was 18.8%. Mean age at time of echocardiogram was 67.4 years (STD 9.7).  Traditional DD risk factors including age, hypertension, coronary artery disease (CAD), renal disease, ever smoker, COPD, and obesity (>= 30kg/m2) were significantly associated with DD on univariable analyses (Table 1). Disease duration, limited cutaneous disease, and presence of anti-Ro52 autoantibody were significantly associated with DD on univariable analysis (Table 1). Only age, CAD, obesity, and a positive anti-Ro52 autoantibody were associated with DD on multivariable model (Table 1). An increase in age-adjusted all-cause mortality was seen in subjects with DD compared to those with normal diastolic function (p<0.0001) (Figure 1).

Conclusion: Patients not only with traditional cardiac risk factors but also with a positive anti-Ro52 autoantibody are at higher risk for DD. Given the poor survival associated with DD in this population, aggressive control of modifiable risk factors as well as further investigation into treatments is warranted.


Disclosure: A. M. Hinze, NIH T32 Research Award, 2; L. K. Hummers, None; F. M. Wigley, Scleroderma Research Foundation and Martha McCrory Professorship, 2; M. Mukherjee, Scleroderma Foundation, 2; A. A. Shah, None.

To cite this abstract in AMA style:

Hinze AM, Hummers LK, Wigley FM, Mukherjee M, Shah AA. Diastolic Dysfunction in Scleroderma: An Investigation into Associated Risk Factors and Impact on Survival [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/diastolic-dysfunction-in-scleroderma-an-investigation-into-associated-risk-factors-and-impact-on-survival/. Accessed March 8, 2021.
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