Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review

摘要: Background The idiopathic systemic capillary leak syndrome is a rare disease characterized by unexplained recurrent shock caused hyperpermeability. Because of the rarity disease, this has easily been misdiagnosed and evidence efficacious agents used empirically lacking. Objective To analyze clinical laboratory data, treatment modalities, mortality rate patients to find contributing factors leading mortality. Methods We searched MEDLINE (inception December 2016) reviewed reference lists previous systematic reviews. A total 133 case reports (161 patients) 5 series (102 patients) were included. Results Patients had hypotension (81.4%), edema (64.6%), flu-like illness (34.2%). This was as hypovolemic shock, septic polycythemia vera, or angioedema. Thirty-seven died (23%) mainly because itself (78.4%). There significant differences in survival rates between who treated with prophylactic β2 agonists, methylxanthines, intravenous immunoglobulins those not. estimated 1-, 5-, 10-year 100%, 94%, respectively. Conclusions systematically analyzed detail presentations all reported identified various associated effects syndrome. findings review will facilitate diagnostic approaches aid selection treatment.