Possible Mediators for Systemic Capillary Leak Syndrome

Nagao, Y.; Harada, Hiromasa; Yamanaka, Hiromi; Fukuda, Ko

doi:10.1016/j.amjmed.2010.04.024

Cited by 16 publications

(10 citation statements)

References 10 publications

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“…However, although direct comparisons with previously reported values are difficult because of methodologic differences in cytokine analysis, the values we measured were considerably lower than those described. 23,24 Neither IL-2 nor IL-8 was elevated in patients with SCLS compared with controls (supplemental Figure 3; data not shown). Given the distinct clinical profile of patients who experienced chronic, continuous symptoms rather than transient, reversible episodes (n ϭ 3), we excluded them from this cytokine analysis.…”

Section: Elevated Vegf and Ang2 Levels In Sclsmentioning

confidence: 99%

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Xie

Ghosh

Patel

et al. 2012

Blood

169

208

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The systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient episodes of hypotensive shock and anasarca thought to arise from reversible microvascular barrier dysfunction. Although the high prevalence of a monoclonal gammopathy of unknown significance in SCLS suggests a pathogenic contribution of endogenous immunoglobulins, the mechanisms of vascular hyperpermeability remain obscure. Herein, we report clinical and molecular findings on 23 patients, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from patients during remission, to human microvascular endothelial cells caused vascular endothelial cadherin internalization, disruption of interendothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin, one promising therapy for SCLS, mitigated the permeability effects of episodic sera. Consistent with the presence of endogenous, nonimmunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Ab-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera.

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Section: Elevated Vegf and Ang2 Levels In Sclsmentioning

confidence: 99%

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Xie

Ghosh

Patel

et al. 2012

Blood

169

208

View full text Add to dashboard Cite

show abstract

“…The patient is at high risk for intravascular volume overload and pulmonary edema during this recruitment phase. The reported cases of the acute phase of SCLS in the literature (from 1990 to 2011, [ 2 , 7 , 8 , 10 - 16 ]) are summarized in Table 1 . Five out of 13 patients had pulmonary edema, which occurred in the recruitment phase in four of them and the other one patient had it in the peripheral leak phase because of the iatrogenic effect of the large amount of transfusion (case 10).…”

Section: Discussionmentioning

confidence: 99%

A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report

et al. 2015

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IntroductionIdiopathic systemic capillary leak syndrome is a rare and fatal disease due to the unexplained episodic attacks of capillary leakage of plasma from the intravascular into the interstitial space. The attack consists of three phases, a prodromal phase, peripheral leak phase and recruitment phase. During the peripheral leak phase, generalized edema, mainly in the trunk and extremities, with hemoconcentration and hypoalbuminemia occurs, while usually the visceral organs like lungs, brain, heart and kidneys seem not to be involved. Treatment of the acute phase is supportive, focusing on adequate but not overzealous fluid resuscitation, because pulmonary edema usually occurs in the recruitment phase.Case presentationA 65-year-old Japanese woman was admitted to our hospital because of severe hypovolemic shock with metabolic acidosis and hemoconcentration and hypoalbuminemia. Although she was considered to be in the peripheral leak phase of idiopathic systemic capillary leak syndrome, which could not be diagnosed during the treatment, the generalized edema worsened further, severe flash pulmonary edema progressed rapidly after fluid resuscitation and she died. The autopsy showed generalized edema, especially alveolar pulmonary edema without endothelial apoptosis.ConclusionsBecause hypovolemic shock and fatal pulmonary edema may progress rapidly together even in the peripheral leak phase of idiopathic systemic capillary leak syndrome, we should keep in mind this rare and fatal disease and recognize the pathophysiology to treat it effectively when the patient has hypovolemia with metabolic acidosis.

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“…Reports suggest that VEGF and angiopoietin 2 (Ang2) contribute to endothelial contraction and might be associated with pathogenesis [12]. Elevated levels of cytokines and chemical mediators (G-CSF, IL-6, IL-8 and monocyte chemotactic protein-1: MCP-1) were reported in some cases [13]. G-CSF and VGEF levels might be a used as biomarkers for the severity and for monitoring the clinical course in ISCLS [14,15].…”

Section: Discussionmentioning

confidence: 99%

10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report

et al. 2014

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BackgroundIdiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by episodic life-threatening hypotension, hypoalbuminemia, and hemoconcentration.Case presentationA 10-year-old girl presented with abdominal pain, vomiting, diarrhea, fever and developed generalized edema a day after admission. Clinical and laboratory findings were consistent with ISCLS. She received aggressive fluid replacement, methylprednisolone pulse (30 mg/kg/day), high-dose intravenous immunoglobulin (IVIG, 2 g/kg/day) and plasma exchange in acute phase. She received fasciotomy of bilateral lower extremities as she developed complications of compartment syndrome. Since there were two episodes of ISCLS attacks, theophylline and terbutaline were initiated for prevention of attacks and then the remission is currently maintained. Because of high fatality rate in ISCLS, prompt diagnosis and intervention are very important.ConclusionWe describe here, a rare case of pediatric ISCLS. ISCLS should be considered as a differential diagnosis, when the patient presents with unexplained or sudden hypovolemic shock. Reports on pediatrics ISCLS are very few, and accumulation of similar case reports is needed.

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Possible Mediators for Systemic Capillary Leak Syndrome

Cited by 16 publications

References 10 publications

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report

10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report

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