Idiopathic capillary leak syndrome or Clarkson’s disease is a rare and potentially life-threatening condition. This condition presents with recurrent crises, characterized by the abrupt onset of generalized edema with relative hypovolemia associated with paradoxical hypoalbuminemia and elevated hematocrit levels. This almost pathognomonic clinical and biological presentation should remind us of the diagnosis, after excluding secondary causes of capillary leaks, and lead to initiating prompt supportive treatment in a specialized department, in an effort to avoid critical care and associated complications. This article describes the clinical characteristics of the disease and current knowledge about its physiopathology, natural history, and long-term prognosis, while highlighting the efficacy of first-line prophylactic therapy using intravenous immunoglobulins (IVIg) for relapse prevention, designed to avoid progression into a more severe form with gloomy prognosis.
Idiopathic capillary leak, Clarkson’s disease, capillary hyperpermeability, monoclonal gammopathy, intravenous immunoglobulins
What is already known about the topic?
Clarkson’s disease is a rare condition with severe prognosis, whose physiopathology remains poorly understood. Currently, this syndrome is thought to be due to a rearrangement of the micro-vascularization, along with a contraction of endothelial cells, mediated by a temporary increase in cytokines and angiogenic factors during the crises.
What does this article bring up for us?
In addition to sharing knowledge about the condition’s signs and symptoms enabling them to be prematurely recognized so that appropriate disease management can be initiated, this article also highlights the efficacy of intravenous immunoglobulins as first-line therapy for patients diagnosed with Clarkson’s disease and monoclonal gammopathy for preventing new crises to occur.