Background/Purpose:

Systemic Sclerosis (SSc) is a complex, heterogenous autoimmune disease characterized by microvascular injury and widespread fibrosis of the skin and internal organs, particularly lungs and heart. The heart is one of the major organs affected in SSc, although the recognition of cardiac abnormalities is probably underestimated due to the occult nature and variable reports of prevalence. Clinically evident cardiac involvement is associated with a poor prognosis, with up to 70% mortality at 5 years. In this population-based cohort study, we aimed to examine the occurrence of moderate/severe valvular heart disease (VHD) in patients with SSc relative to comparators.

Methods:

Medical records of patients with a diagnosis or suspicion of SSc in a geographically well-defined area from Jan 1, 1980 to Dec 31, 2016 were reviewed to identify incident cases of SSc (defined by physician diagnosis). Fulfillment of the 1980 and 2013 SSc classification criteria was ascertained. A 2:1 cohort of age- and sex-matched non-SSc subjects from the same population base was randomly selected for comparison. Data on SSc characteristics, cardiovascular risk factors including smoking status, obesity, hypertension, dyslipidemia, diabetes mellitus, use of aspirin, known coronary heart disease, peripheral artery disease, abdominal aneurysm, atrial fibrillation, and heart failure were collected by manual record review. Echocardiogram reports were reviewed for occurrence of moderate/severe VHD including aortic, pulmonary, mitral or tricuspid valve stenosis or regurgitation. Cumulative incidence of VHD adjusting for the competing risk of death was estimated. Cox models were used to examine potential associations between baseline factors of interest and the development of VHD.

Results:

The study included a total of 79 incident SSc cases and 158 non-SSc comparators [mean age 55.8 years (SD 15.7), 90% female for both cohorts]. There was a nearly 4 fold increase in the prevalence of moderate/severe VHD prior to SSc diagnosis compared to non-SSc subjects (11% vs 3%; p=0.011). During a median of 9.0 years of follow-up in patients with SSc and 10.5 years of follow-up in non-SSc comparators, 15 SSc and 15 non-SSc patients developed VHD. The cumulative incidence of VHD at 10 years after SSc incidence/index was 23.6% (95% confidence interval [CI]: 14.5-38.4%) in patients with SSc compared with 7.4% (95% CI: 4.0-13.5) in non-SSc subjects (Hazard Ratio: 2.86; 95%CI: 1.38- 5.91, p=0.004). No significant risk factors for VHD were found.

Conclusion:

SSc patients have a 3 fold increased risk of developing moderate/severe valvular dysfunction after diagnosis of SSc compared to Non-SSc subjects. They also have a 4 fold increased prevalence of moderate-severe VHD at diagnosis compared to non-SSc patients. Underlying mechanisms for this association require further elucidation.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/increased-risk-of-valvular-heart-disease-in-patients-with-systemic-sclerosis-results-from-a-population-based-cohort-1980-2016/