[Idiopathic and secondary capillary leak syndromes: A systematic review of the literature]

The capillary leak syndrome (CLS) is a rare condition characterized by the onset of hypotension, edema, hemoconcentration and hypoalbuminemia. CLS can be idiopathic (Clarkson's disease) or secondary to various conditions and treatments. Here, we review the clinical and biological features, pathophysiology, causes and treatment of this rare condition.

We performed a systematic review of the literature (Medline database through February 2014) to identify all articles about CLS. The relevant references were selected by two independent authors.

Secondary CLSs are mostly due to malignant hematological diseases, viral infections, and treatments such as chemotherapies and therapeutic growth factors. Diagnosis of idiopathic CLS is made by exclusion of secondary diseases, especially as a serum monoclonal immunoglobulin is present, or when there is a relapsing disease, no initial lung involvement or preserved consciousness despite low blood pressure. Acute episodes are treated with vasopressor therapy and judicious fluid replacement. Between episodes, patients with Clarkson's disease may be treated with intravenous immunoglobulins. CLS is a severe disease with significantly impaired prognosis.

Clarification of the pathophysiological mechanisms of CLS is essential to improve the prognosis of this rare disease with more targeted treatments.