Clarkson’s Disease - Capillary leak syndrome

Epidemiology: Roughly 150 published cases, Median age 50 years, No sex predominance, Relapsing-remitting course +++

Clinical features:

  1. Hypovolemia: Asthenia, lipothymia, thirst, hypotension, tachycardia, oliguria

  2. Leakage of fluids into tissues: Myalgia, paresthesia, abdominal pain, Diffuse edema, compartment syndrome

Laboratory features: 

  1. Hemoconcentration: elevated hematocrit, leukocytosis

  2. Paradoxical hypoalbuminemia with no urine protein excretion

3. Monoclonal gammopathy (IgG+++)

Treatments - To treat disease flares: 

 • No effective treatment (no IVIg)

 • Limit vascular filling +++ (risk of pulmonary edema)

Treatments - To prevent disease flares:

 • Prevention of at-risk situations (vaccines, treatment of infections)

 • IVIg 2 g/kg/month at least 1 year

 • Discuss spacing of IVIg cures



By Dr. Paul Breillat 



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