Objective

To determine the independent risk factors for diastolic dysfunction (DD) in patients with systemic sclerosis (SSc) and to evaluate the impact of DD on mortality.

Methods

SSc patients enrolled in the Johns Hopkins Scleroderma Center Cohort between 11/01/2006 through 11/01/2017 with at least one analyzable 2D echocardiogram (2DE) in our system were included, totaling 806 participants. DD risk factors and SSc disease characteristics were prospectively obtained, and presence/absence of DD was determined by the most recent 2DE. Logistic regression models examined associations between clinical risk factors and DD, and Cox proportional hazards models were used to assess survival.

Results

DD was present in 18.6% of participants. The majority of participants were female (84%) with a median age of 58.4 years (IQR 48.8, 68.1). Older age (OR 1.12, 95%CI 1.09-1.15), coronary artery disease (OR 3.69, 95%CI 1.52-8.97), obesity (4.74, 95%CI 2.57-8.74), longer SSc disease duration (OR 1.04, 95%CI 1.01-1.06), diffusing capacity (DLCO) ≤60% of predicted (OR 2.41, 95%CI 1.40-4.16), and history of scleroderma renal crisis (OR 3.18, 95%CI 1.12-9.07), were all independently associated with an increased risk of DD. Anti-Scl70 positivity (OR 0.49, 95%CI 0.26-0.93) and severe gastrointestinal disease (OR 0.48, 95%CI 0.30-0.79) were associated with a reduced risk of DD. The presence of DD was independently associated with an increase in mortality (HR 1.69, 95%CI 1.07-2.68).

Conclusion

DD is independently associated with an increased risk of mortality in patients with SSc. Potentially modifiable risk factors, including CAD and obesity, should be addressed in patients with SSc to reduce mortality risk.