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The Idiopathic Systemic Capillary Leak Syndrome (ISCLS), also known as Clarkson’s syndrome, is a rare and serious medical condition characterized by recurrent episodes of sudden leakage of fluid from small blood vessels (capillaries) in the body. This leads to abnormal fluid accumulation in tissues (edema) and a drastic reduction in blood volume (hypovolemia), resulting in shock, organ failure, and, in extreme cases, risk of death.
The exact cause of ISCLS is unknown (idiopathic), and it is not associated with any visible damage to the vessels. Episodes of capillary leakage can be triggered by infections, physical stress, or other factors. Symptoms range from muscle pain, fatigue, swelling, to severe hypotension. The condition is extremely rare and requires early diagnosis and rapid treatment aimed at minimizing the complications of shock while reducing, where possible, the amount of infused fluids.
It is crucial that patients diagnosed with ISCLS are treated at a center specialized in managing this medical condition. To date, the only treatment agreed upon in medical literature is preventive, aimed at avoiding relapses, and involves the periodic infusion of polyvalent human immunoglobulins.
The exact cause of ISCLS is unknown (idiopathic), and it is not associated with any visible damage to the vessels. Episodes of capillary leakage can be triggered by infections, physical stress, or other factors. Symptoms range from muscle pain, fatigue, swelling, to severe hypotension. The condition is extremely rare and requires early diagnosis and rapid treatment aimed at minimizing the complications of shock while reducing, where possible, the amount of infused fluids.
It is crucial that patients diagnosed with ISCLS are treated at a center specialized in managing this medical condition. To date, the only treatment agreed upon in medical literature is preventive, aimed at avoiding relapses, and involves the periodic infusion of polyvalent human immunoglobulins.
During acute ISCLS crises, adherens complexes between endothelial cells undergo a conformational change. This allows proteins, which are normally contained within the capillary, to pass into the extravascular space. During these attacks, proteins with a molecular weight of up to 200-300 kDa freely pass through the inter-endothelial spaces, while proteins with a higher molecular weight, such as IgM (900 kDa), apparently remain into the vascular bed.
