Systemic capillary leak syndrome

The systemic capillary leak syndrome describes a rare systemic disease caused by increased capillary permeability.

The age of onset in approximately at the age of 45. Patients suffer from periodic dormancy and crisis after the first appearance of the symptoms. Partial rest periods of several years have been observed. 

A crisis can be triggered due to an infection or severe physical stress. The main symptoms are hypotension, edemas and hypovolemia. The crises may vary in severity, with symptoms ranging from tiredness and a runny nose to arrhythmia and thrombosis.

The cause to hypermeability of the capillaries is yet unclear. Possible causes to this include damages to the capillary endothelium or inflammation-mediated mechanisms. 

The prognosis is unsatisfactory, and about a third of the patients die after 10 years.