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

@article{Xie2012VascularEH,
  title={Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome).},
  author={Zhihui Xie and Chandra C. Ghosh and Roshni B. Patel and Shoko Iwaki and Donna Gaskins and Celeste Nelson and N Jones and Philip R. Greipp and Samir M. Parikh and Kirk M. Druey},
  journal={Blood},
  year={2012},
  volume={119 18},
  pages={
          4321-32
        }
}
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… Expand
Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome
TLDR
Results demonstrate that ET-1 triggers classical mechanisms of vascular barrier dysfunction in ECs through ETA, and a link between SCLS and endothelial ETA expression is suggested. Expand
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease)
TLDR
The presence of proinflammatory cytokines in acute SCLS sera suggests that inflammation or infection may have a role in triggering episodes, and the enhanced capacity of monocytes from SclS patients to produce CXCL10 suggests a new therapeutic avenue for SCLs. Expand
Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report
TLDR
In this report, a case of systemic capillary leak syndrome is described in which serum isoform D of vascular endothelial growth factor was elevated, which was normalized after the introduction of thalidomide. Expand
Neutrophil activation in systemic capillary leak syndrome (Clarkson disease)
TLDR
The results suggest systemic neutrophil degranulation during SCLS acute flares, which may contribute to the clinical manifestations of acute vascular leak. Expand
Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome
TLDR
Patients with active SCLS have significantly higher myocardial ECV than age-matched controls or SclS patients in remission, which correlated with histopathological findings in one patient. Expand
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson’s Disease)
TLDR
This case report supports the view that damage to the microvascular endothelium, has a role in the pathogenesis of acute SCLS and indicated that monitoring serum levels of syndecan-1 (CD138) might be used to monitor the progression and resolution of episodes of SCLs. Expand
Idiopathic Systemic Capillary Leak Syndrome (Clarkson Disease)
TLDR
This chapter outlines the diagnostic dilemmas and current approaches related to the management of idiopathic SCLS, a disorder with myriad non-specific prodromal and acute attack-related symptoms. Expand
Idiopathic Systemic Capillary Leak Syndrome in Children
TLDR
Prophylactic medication, including high dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy. Expand
Clinical and Pathological Findings of a Fatal Systemic Capillary Leak Syndrome (Clarkson Disease)
AbstractSystemic capillary leak syndrome (SCLS) is a rare disorder with episodes of hypotension, hypoalbuminemia, and hemoconcentration. During attacks endothelial hyperpermeability results inExpand
Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease.
TLDR
These results support the previous hypothesis that increased VEGF levels mark the onset of acute SCLS flares and correlate with episode severity, and support the development of molecular biomarkers to aid diagnosis. Expand
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It is concluded that the patient had the chronic orm of capillary leak syndrome, a rare disorder charcterized by acute attacks of severe vascular hyperpermebility causing hypotension and shock, by exclusion of other causes. Expand
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The pathology of the same patient, who died during an attack, is studied and it is found that in the skin there were perivascular infiltrates of CD8 lymphocytes surrounding damaged endothelial cells, a picture similar to that of r IL-2-dependen t capillary leak syndrome (CLS). Expand
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Components from the sera of patients with active systemic capillary leak syndrome in contrast to healthy subject sera mediate early and extensive endothelial apoptosis in vitro that is associated with oxidation injury, providing compelling initial evidence for oxidation-induced apoptosis as a likely mechanism for endothelial injury leading to SCLS. Expand
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Clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension, and patients who survive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%. Expand
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The microvessels of skeletal muscle were examined electron microscopically in a case of systemic capillary leak syndrome and the blebbing is tentatively interpreted as a sign of a complement-mediated injury to the endothelium, leading to a breakdown of the endothelial barrier. Expand
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An improvement in the prognosis of SCLS is shown due most likely to improved management during attacks, and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy. Expand
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