Research progress of idiopathic systemic capillary leakage syndrome / 中国中西医结合急救杂志

ABSTRACT

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease, the clinical manifestation is increased acute episodic capillary leakage (SCL), leading to protein-rich fluid leakage from the blood vessels to the interstitial tissue space, resulting in progressive systemic edema, hypoproteinemia, hypotension, blood concentration and other clinical symptoms. Because of ISCLS frequent recurrences, high mortality, pathogenesis unclear and no definite treatment plan, it is extremely important to fully understand the disease to carry out early intervention to save the patients' lives. This article reviews the progress of ISCLS, including its disease onset characteristics, clinical features, pathological mechanism, diagnosis and treatment of ISCLS.