Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

Kinoshita, Yoshiaki; Kasaoka, Shunji; Fujita, Motoki; Oshima, Chiyomi; Kawamura, Y.; Tsuruta, Ryosuke; Maekawa, Taira

doi:10.2169/internalmedicine.49.2929

Cited by 18 publications

(19 citation statements)

References 15 publications

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“…In agreement with individual case reports, our patients had elevated serum VEGF levels at the onset of an acute SCLS episode compared with their baseline, and in one patient VEGF returned to baseline rapidly on presentation with a severe hypotensive crisis. 10,22 Our results show that a short interval of elevated VEGF (and possibly other permeability factors) may exist in SCLS. , and anti-VEGF (bevacizumab) or equivalent concentrations of control IgG (for anti-Ang2 and bevacizumab) or BSA (for IVIG) against episodic sera from 4 to 6 patients was evaluated in the TEER assay.…”

Section: Discussionmentioning

confidence: 63%

“…20,21 A recent case report found acutely elevated serum VEGF in one patient with SCLS experiencing active symptoms, which correlated with the clinical course. 22 In our series of 20 patients with classic acute SCLS, serum VEGF and Ang2 levels were significantly elevated in the SCLS group during acute episodes compared with asymptomatic SCLS patients and healthy controls (Figure 5A-B). Notably, serum Ang2 was also elevated in patients with SCLS during asymptomatic periods relative to the control group (Figure 5B), which may render them more susceptible to vascular leakage.…”

Section: Elevated Vegf and Ang2 Levels In Sclsmentioning

confidence: 71%

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

Xie

Ghosh

Patel

et al. 2012

Blood

173

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: Discussionmentioning

confidence: 63%

Section: Elevated Vegf and Ang2 Levels In Sclsmentioning

confidence: 71%

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

Xie

Ghosh

Patel

et al. 2012

Blood

173

208

View full text Add to dashboard Cite

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“…Vascular endothelial growth factor (VEGF) is involved in endothelial homeostasis and permeability and there is evidence of a relationship between serum VEGF and the clinical course of SCLS 3. In this respect, SCLS has similarities to the POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, myeloma and skin abnormalities) which is mediated through VEGF and is associated with plasma cell disorders.…”

Section: Discussionmentioning

confidence: 99%

Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

et al. 2013

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A female patient in her 60s presented with a history of malaise, chills, headache and vomiting. She was in shock on presentation with a high haematocrit and a low albumin with evidence of rhabdomyolysis. Severe limb and truncal oedema developed with worsening hypotension leading to intensive care unit admission for multiple organ support. Extensive radiological, microbiological and immunological work up was negative with the exception of a monoclonal gammopathy. A review of patient investigations led to a diagnosis of Clarkson's disease. Treatment with high-dose methylprednisolone and intravenous immunoglobulins led to a rapid decline in the creatine kinase (CK) level and vasopressor requirements. The patient was discharged home on long-term terbutaline and has made a good recovery.

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“…Serum levels of complements, kinins, prostaglandin, coagulation factors, histamine and serotonin are usually normal in patients with SCLS. Activation of the 5-lipoxygenase pathway and a role for IL-2, VEGF and interferon-β has been suggested (9)(10)(11)(12). It has also been reported that SCLS occurs after infusions of IL-2, IL-4, tumor necrosis factor, granulocyte-macrophage colony-stimulating factor (GM-CSF) and granulocyte colony-stimulating factor (G-CSF) as well as after autologous and allogeneic stem cell transplantation (13)(14)(15).…”

Section: Discussionmentioning

confidence: 99%

A Case of Idiopathic Systemic Capillary Leak Syndrome with High Serum Levels of G-CSF on Exacerbation

et al. 2011

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Systemic capillary leak syndrome (SCLS) is a life-threatening disorder which presents with periodic episodes of hypovolemic shock, due to plasma leakage to the extra-vascular space reflected by accompanying hypoalbuminemia, hemoconcentration and edema often with associated monoclonal gammopathy. We describe a 28-year-old woman with SCLS who required aggressive fluid resuscitation and was successfully treated with corticosteroid, terbutaline, and theophylline. At exacerbation, the levels of serum granulocyte colony-stimulating factor (G-CSF) were increased. Thus, G-CSF might play an important role and can be a useful biomarker for the severity of attacks in SCLS.

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Synchronized Changes in Serum Vascular Endothelial Growth Factor during the Clinical Course of Chronic Systemic Capillary Leak Syndrome

Abstract: Abstract

Cited by 18 publications

References 15 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)

Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

A Case of Idiopathic Systemic Capillary Leak Syndrome with High Serum Levels of G-CSF on Exacerbation

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