COVID-19 and systemic sclerosis: Rising to the challenge of a pandemic

Abstract

COVID-19, caused by infection of the novel coronavirus SARS-CoV-2, has caused a pandemic of enormous impact that has challenged healthcare and political system throughout the world. This new health emergency has occurred on top of the usual burden of diseases, including systemic sclerosis, and has led to many unanticipated consequences. An early consequence of the pandemic was postponement of the Sixth Systemic Sclerosis World Congress that was recently completed as a successful virtual congress with more than 1000 delegates. In this article, we summarise the relevance and impact of COVID-19 from the perspective of systemic sclerosis. Shared concepts of pathogenesis are considered, and the relevant literature emerging about COVID-19 and systemic sclerosis summarised. The specific impact of this pandemic on delivery of optimal scleroderma care is considered, together with the broader effect on rheumatic and musculoskeletal diseases and the activities of European League Against Rheumatism. As the World continues to struggle against this new infectious disease, it is notable that expertise and growing understanding of systemic sclerosis has been able to help tackle COVID-19. Moreover, the essential adjustments to deliver clinical care and establishment of new ways of working due to the pandemic have offered potential avenues for future improvement in systemic sclerosis care.

Keywords: COVID-19; EULAR; lung fibrosis; pathogenesis; scleroderma.

Figure 1.

Shared clinical features of systemic sclerosis and COVD-19. There are shared clinical features between systemic sclerosis and COVID-19, including mixed patterns of parenchymal and vascular lung disease, myocardial abnormalities with inflammation and systemic peripheral vasculopathy as evidenced by lesions of the toes, in this case with perniosis (so-called ‘Covid toe’). All clinical images are from patients with COVID-19.

Figure 2.

Conceptual framework for aetiopathogenesis of systemic sclerosis and COVID-19: The development of clinical disease depends upon three factors. Susceptibility of the individual, the appropriate environmental trigger or series of triggers that lead to disease and random chance that determines whether the trigger meets the host at an appropriate time to cause disease. (a) For COVID-19 clearly the trigger is SARS-CoV-2 the trigger, but a multitude of host factors determine the timing, dose, and pattern of exposure. Host factors are highly likely to be the major determinant of disease phenotype and clinical outcome. (b) Analogy can be drawn with a complex chronic disease like systemic sclerosis. Here multiple triggers over time are likely to be required and lead to progression from pre-disease state to established diagnosis. Host factors are also likely to be major determinants of disease phenotype, severity, and outcome. It is possible that immunogenomic factors determining susceptibility to tissue damage and poor outcome are shared between the autoimmune landscape of SSc and the virally triggered immuno-inflammation of COVID-19.