Methods: A total of 754 subjects with scleroderma seen between November 1^st, 2007 through October 31^st, 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.