Systemic sclerosis (SSc) is an autoimmune disorder characterised by the buildup of scar tissue in the skin and organs, particularly the lungs, heart and digestive tract. Heart involvement is a severe complication of SSc, with a reported five-year mortality of 70%. The risk of valvular heart disease (VHD) in SSc is still an underrecognised cardiac complication and it has been suggested that SSc patients have a fourfold increase in the risk of moderate or severe VHD compared to non-SSc cases. As SSc cases continue to rise in the world, the added risk of VHD complications will add significant challenges to the management of SSc.
According to a May 2021 study conducted by Kurmann and colleagues and published in The Journal of Rheumatology, 24% of SSc patients had VHD, compared with 5% in the non-SSc population. SSc patients also had a higher prevalence of moderate to severe VHD compared with non-SSc patients. This suggests that SSc patients not only have a higher prevalence of VHD, but these heart conditions are also much more serious and life-threatening in these patients.
GlobalData epidemiologists forecast that the diagnosed prevalent cases of SSc in the seven major markets (7MM, namely the US, France, Germany, Italy, Spain, UK and Japan) will grow at an annual growth rate (AGR) of 1% a year over the next ten years, from 170,000 cases last year to 190,000 cases in 2030 (Figure 1). GlobalData epidemiologists also reported that the number of cases with heart conditions in SSc was 24,000 last year in the 7MM.
Kurmann and colleagues have reported that heart conditions are underdiagnosed in SSc, especially in the early stages. Heart conditions in SSc are underestimated, as these conditions remain subclinical with no overt signs and symptoms until the disease progresses to advanced stages. As the involvement of the heart condition is associated with a poor prognosis and is a strong predictor of mortality, early detection and monitoring of heart conditions is very important in managing the SSc patient population.