ArticleBlood2012
Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome).
Article in Blood, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 107 papers.
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Who cites it
107 citing papers in PubMed, 190 citations in OpenAlex.
- Idiopathic systemic capillary leak syndrome and related shock in PICU: an underdiagnosed disease?Journal of anesthesia, analgesia and critical care · 2026Article
- Emergency in pediatric rheumatology: a narrative review.Frontiers in medicine · 2026Review
- Allogeneic haematopoietic cell transplantation promotes atherosclerosis in mice via CD8+ T cells.Cardiovascular research · 2025Article
- An immune competent in vitro model of IL2 induced vascular leakage enables mechanistic insights and evaluation of mitigation strategies.Scientific reports · 2025Article
- A 74-Year-Old Man with Refractory Hypotension in the Setting of COVID-19 Infection.Annals of the American Thoracic Society · 2025Article
- Systemic capillary leak syndrome: a nosological entity that the nephrologist must be aware of.Journal of nephrology · 2025Review
- Clarkson disease in critically and non-critically ill patients: insights from the Italian IRIS-CLS registry.Internal and emergency medicine · 2025Article
- Alveolar Bile and Light Chain Immunoglobulin Depositions as an Unusual Complication of Transjugular Liver Biopsy Resulting in Bilhemia in a Patient with Multiple Myeloma.Journal of clinical medicine · 2025Article
- Clinical application of acute Q fever -induced systemic capillary leak syndrome in a patient by using metagenomic next-generation sequencing: a case report and literature review.BMC infectious diseases · 2025Review
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- Systemic capillary leak syndrome.Nature reviews. Disease primers · 2024Review
- Capillary Leak Syndrome Associated with Anaplastic T Cell Lymphoma and Transcutaneous Exudation: An Unusual Presentation.Diagnostics (Basel, Switzerland) · 2024Article
- Hypersensitive blood vessels in Clarkson disease.The Journal of clinical investigation · 2024Article
- Idiopathic Capillary Leak Syndrome (Clarkson's Disease): First Reported Case in Oman.Oman medical journal · 2024Article
- Intrinsic endothelial hyperresponsiveness to inflammatory mediators drives acute episodes in models of Clarkson disease.The Journal of clinical investigation · 2024Article
- Ultrasound findings and specific intrinsic blood volume expansion therapy for neonatal capillary leak syndrome: report from a multicenter prospective self-control study.European journal of medical research · 2024Article
- Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature.Oncology and therapy · 2024Article
47 more citing papers are in PubMed but not listed here.
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Authors and funding
10 authors at 5 institutions in 1 country.
Funding
Abstract
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. Comparable experiments with anti-VEGF Ab (bevacizumab) yielded less interpretable results, probably because of endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which nonimmunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.