Background/Purpose:

Scleroderma or systemic sclerosis (SSc) is an autoimmune disease characterized by microangiopathy, tissue hypoxia, and fibrosis. At least seven different autoantibodies have been identified with distinct phenotypic associations. A recent study involving patients with limited and diffuse SSc found the prevalence of diastolic dysfunction to be 23%.[1] Serious cardiac involvement defined as symptomatic heart failure, arrhythmia, or pericardial effusion, has been associated with Scl-70 antibodies when compared to RNA polymerase III (Pol-III) positive patients.[2]The aim of this study was to compare prevalence of subclinical diastolic dysfunction in diffuse SSc patients with Pol-III antibodies or Scl-70 antibodies.

Methods:

Forty two patients from a major tertiary referral center with known SSc and Pol-III antibodies provided a cohort of 18 patients after the exclusion of those with limited disease. Similarly, 540 patients diagnosed with SSc were screened for Scl-70 antibodies providing a cohort of 24 patients after the exclusion of those with limited disease phenotype. Demographic and echocardiographic indices for diastology were collected.  Welch’s two-sample t-test and Pearson’s chi-squared test were used for continuous and categorical variables, respectively. Logistic regression was used to compare binary measurements and linear regression models for continuous measurements after adjusting for disease duration. A significance level of 5% was used for all analyses.

Results:

By unadjusted analysis, 26% of the Pol-III patients had diastolic dysfunction, compared to 74% of the Scl-70 group. This difference reached statistical significance with a p-value of 0.035. After adjusting for disease duration, the estimated odds ratio of having diastolic dysfunction was 4.2 times higher for Scl-70 patients than for Pol-III patients. Although the latter finding did not reach statistical significance, it can be thought of as a trend with a p-value of 0.052. There was no evidence that the septal E/E’ ratio, lateral E/E’ ratio, E/A ratio, or left ventricular mass index differ between the two groups.

Conclusion:

Diastolic dysfunction is prevalent amongst SSc patients with diffuse disease. Scl-70 and Pol-III antibodies are associated with diastolic dysfunction in patient with diffuse SSc. After adjusting for disease duration, Scl-70 patients had a proportionally higher prevalence of diastolic dysfunction than Pol-III patients with a trend towards significance. These autoantibodies may be markers of risk for developing myocardial dysfunction.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/diastolic-dysfunction-amongst-autoantibody-subgroups-of-patients-with-diffuse-scleroderma/