SARS-CoV-2-induces acute and refractory relapse of systemic capillary leak syndrome (Clarkson’s disease)

The systemic capillary-leak syndrome (SCLS), also known as Clarkson’s disease, is a rare condition characterized by recurrent episodes of capillary hyperpermeability in the context of a monoclonal gammopathy1. We have previously shown that prophylactic treatment with intravenous immunoglobulins (IVIg) significantly reduces relapse and improve survival2,3. SCLS episodes are thought to have an infectious trigger, especially mediated by viruses and a flu-like viral syndrome was reported in more than half of the episodes in a large cohort of Clarkson’s disease flares4.