SARS-CoV-2-induces acute and refractory relapse of systemic
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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 gammopathy.

A 45-year-old woman, with a 7-year history of IgG Kappa monoclonal gammopathy-associated SCLS, was recently admitted to the hospital for a planned immunoglobulin infusion. She regularly received IVIg since her diagnosis of Clarkson's disease at an initial dosage of 2g/kg, with progressive tapering to 0.5g/kg of body weight monthly.

When she was admitted in March 2020 she complained about nausea and vomiting and a 10 kg increase of body weight. She had hypotension with elevated heart rate. Laboratory findings were typical for an acute episode. Evolution was unfavorable with severe hypovolemic shock, multiple organ failure and 4-limb compartment syndrome cumulating into refractory cardiac arrest. SARS-CoV-2 PCR turned out to be positive as sampled by tracheal aspiration.

The pathophysiology of SCLS is unknown. Whether the monoclonal component contributes to the pathogenesis of the disease and the mode of action of IVIg remains elusive. Still, the role of viruses as a trigger for acute relapses of the disease has repeatedly been described, as holds particularly for the influenza virus. SARS-CoV-2, a novel coronavirus that spread in early 2020 from the region of Wuhan in China is characterized mainly by a severe acute respiratory distress syndrome. In this patient, the viral infection, although poorly symptomatic, can be considered as the trigger of the relapse.

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