Capillary leakage syndrome: a case report and a review

Capillary leakage Syndrome (CLS) is a rare clinical syndrome, that was first described in 1960, characterized by acute episodes of generalized edema, hemoconcentration, hypoproteinemia and monoclonal gammopathy, in the vast majority of cases. We describe a 39-year-old man with anasarca, bilateral pleural and pericardial effusions, ascites and diffuse alveolo-intersticial edema. Clinical and laboratory findings were consistent with an acute episode of CLS. Treatment with prednisone, furosemide and aminophylline was started, which lead to a gradual improvement in 48 hours. Pathophysiologically there is an increase in capillary permeability with the extravasation of fluid and plasmatic proteins to the extravascular space that can lead to hypovolaemic shock. In the second phase there is a reentry of the fluid overload leading to pulmonary edema. The etiology of this hyperpermeability still remains unclear. The role of cytokines has become central in the comprehension of pathophysiology of CLS. Adhesion molecules are probably also involved in the genesis of capillary leakage. CLS treatment remains empirical. However, at present it seems that the association of steroids with furosemide, aminophylline and terbutaline are capable of controlling the clinical manifestation of the acute episodes in most cases. To our knowledge no prophylatic therapy has clearly proven its efficacy. There are only a few series analyzing the long-term evolution of patients with CLS. Further studies are necessary with the objective to collect enough patients with CLS to observe natural history of the disease and evaluate the efficacy of empiric treatments.