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 #Clarksons #Disease #Capillary #leak #syndrome #diagnosis #management #timeline #treatment #rheumatology
