Sudden Cardiac Death in Systemic Sclerosis: Diagnostics to Assess Risk and Inform Management

Cardiac disease is a leading cause of death in systemic sclerosis (SSc) and sudden cardiac death (SCD) is thought to occur more commonly in SSc than in the general population. Diffuse myocardial fibrosis, myocarditis and ischaemic heart disease are all prevalent in SSc and can be reasonably hypothesised to contribute to an increased risk of SCD. Despite this, SCD remains a relatively understudied area of SSc with little understood about SSc-specific risk factors and opportunities for primary prevention. In this review, we present an overview of the possible mechanisms of SCD in SSc and our current understanding of how each of these mechanisms may contribute to cardiac death. This review highlights the need for a future research agenda that addresses the underlying epidemiology of SCD in SSc and identifies opportunities for intervention to modify the disease course of heart disease in SSc.

Automated summary

Cardiac disease in systemic sclerosis (SSc) is a leading cause of death, with sudden cardiac death (SCD) occurring more commonly in SSc patients. Diffuse myocardial fibrosis, myocarditis, and ischemic heart disease are prevalent in SSc and contribute to the increased risk of SCD. Further research is needed to understand the specific risk factors for SCD in SSc and identify opportunities for primary prevention.