35 Systemic capillary leak syndrome

Capillary leak syndrome (CLS) is a rare, life-threatening disorder characterized by recurrent episodes of fluid leaking from the blood vessels into the surrounding tissues, hypotension, oedema, haemoconcentration and hypoalbuminemia.¹ CLS can be idiopathic (Clarkson's disease) or secondary to various conditions and treatments.¹ Secondary CLS typically results from, systemic autoimmune disorders, viral infections, malignant haematological diseases and treatments such as chemotherapies and therapeutic growth factors.¹ 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.¹ Prophylactic treatment of Clarkson's disease with intravenous immunoglobulin may reduce the frequency and severity of attacks and may improve survival.

Reference

Duron L, et al. Syndrome de fuite capillaire idiopathique et formes secondaires : une revue systématique de la littérature [Idiopathic and secondary capillary leak syndromes: A systematic review of the literature]. Rev Med Interne. 2015 Jun;36(6):386–94.

Learning Objectives

Describe the clinical presentation of systemic CLS, including symptoms, signs, and complications Discuss the diagnosis of systemic CLS, including diagnostic tests and criteria. Discuss treatment options for systemic CLS, including both acute and chronic management