Systemic capillary leak syndrome (SCLS) is a rare condition characterized by episodic capillary hyperpermeability. The manifestations of this syndrome are generalized edema, hypotension, hypoalbuminemia, hemoconcentration, renal shut-down and monoclonal gammopathy. The pathogenesis of SCLS is still unclear. The treatment of SCLS is empirical, including theophylline, terbutaline, steroids, loop diuretics, plasmapheresis and BMT. Some patients were diagnosed multiple myeloma during follow-up. Death during the acute attack from cardiopulmonary collapse or diuresis phase from pulmonary edema occurs in approximately one third of the cases. Therefore initial fluid therapy is one of critical care point, but there is no information what is the adequate volume expander during acute attack. We experienced two cases of SCLS with severe acute attack and showed rapid improvement of hypotension by 10% pentastarch.

Case 1: A 36-year-old woman visited our hospital with systolic blood pressure (SBP) 50 mmHg, resting dyspnea, oliguria and anasarca. The laboratory findings showed Hb 19.2 g/dL, hematocrit (Hct) 53%, total protein / albumin 3.7 / 1.7 g/dL, BUN / creatinine 32.6 / 1.9 mg/dL and monoclonal IgG-kappa. There was no evidence of multiple myeloma. We diagnosed the patient as having SCLS and started intravenous infusionof 9 L of normal saline (NS), dexamethasone, aminophylline and inotropics for 8 hours. Despite massive fluid therapy, SBP was not increased, and respiratory failure due to pulmonary edema was developed. After infusion of 10% pentastarch 0.5 L every 8 hours, SBP was gradually increased to normal within 12 hours without NS infusion. From the next day, diuresis phase started, other clinical and laboratory findings became normal range. There was no long-term sequale, and no more attack for 1 year follow-up.

Case 2: A 36-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed typical SCLS with the presence of monoclonal IgG-kappa. There was no evidence of multiple myeloma. She had histories of similar several episodes for 3 years, was managed with infusion of albumin, NS and suffered from pulmonary edema. At this time, clinical and laboratory findings were severe: confusion, anuria, SBP 40 mmHg, Hb 21.1 g/dL, Hct 63%, BUN / creatinine 22.6 / 2.8 mg/dL, total protein / albumin 4.4 / 2.5 g/dL. Two hours after using 0.5 L of 10% pentastarch and 2 L of NS, SBP and urine output increased to 127 mmHg and 50 ml/hour, respectively. Other clinical and laboratory findings restored and diuresis phase started within 1 day and there was no pulmonary edema.

Acute management during hypotensive attack is very important to reduce mortality of SCLS. Because shift of fluid and protein by capillary hyperpermeability is the pathophysiology of this syndrome, total body fluid is not decreased. Thus massive crystalloid fluid, albumin infusion would not effective during hypotensive phase of SCLS and exacerbate edema. Hypoalbuminemia (molecular weight (MW) 69,000 dalton) is very common manifestation of SCLS, and

Atkinson et al (
reported that egress from vascular compartment was limited to proteins of MW up to 200,000 dalton. Under this knowledge, we used pentastarch (MW 240,000 dalton) during acute SCLS attacks and it showed dramatic responses. Pentastarch would be a very effective treatment for acute attack and might decrease acute mortality of SCLS.

Disclosure: No relevant conflicts of interest to declare.

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