Chihara Ruri, Nakamoto Hidetomo, Arima Hiroshi, Moriwaki Kenshi, Kanno Yoshihiko, Sugahara Soichi, Okada Hirokazu, Suzuki Hiromichi
Department of Nephrology, Saitama Medical School, Iruma-gun, Saitama.
Intern Med. 2002 Nov;41(11):953-6. doi: 10.2169/internalmedicine.41.953.
Systemic capillary leak syndrome (SCLS) is characterized by recurrent hypovolemic shock attributable to increased systemic capillary leakage. A 46-year-old man was admitted to our hospital because of recurrent episodes of generalized edema with hypovolemic shock. Blood laboratory data revealed severe hypoproteinemia with a small monoclonal IgG-kappa protein. These findings suggested strongly the diagnosis of SCLS. A regimen with steroid pulse therapy was tried; however, intravascular overloading accompanied by the recruitment of the initially extravasated fluids resulted in acute pulmonary edema. Intensive care and careful monitoring of fluid volume are required in SCLS.
系统性毛细血管渗漏综合征（SCLS）的特征是由于全身毛细血管渗漏增加导致反复出现低血容量性休克。一名46岁男性因反复出现全身性水肿伴低血容量性休克而入住我院。血液实验室检查数据显示严重低蛋白血症伴少量单克隆IgG-κ蛋白。这些发现强烈提示SCLS的诊断。尝试了类固醇脉冲疗法；然而，血管内液体超负荷并伴有最初渗出液体的回吸收导致了急性肺水肿。SCLS患者需要重症监护并密切监测液体量。
