ArticleJournal of clinical & cellular immunology2014
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease).
Article in Journal of clinical & cellular immunology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers, 1 of them a synthesis that pooled it.
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Who cites it
43 citing papers in PubMed, 1 synthesis or guideline pooled it, 69 citations in OpenAlex.
- Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.European journal of pediatrics · 2018Pooled it
- Idiopathic systemic capillary leak syndrome and related shock in PICU: an underdiagnosed disease?Journal of anesthesia, analgesia and critical care · 2026Article
- An immune competent in vitro model of IL2 induced vascular leakage enables mechanistic insights and evaluation of mitigation strategies.Scientific reports · 2025Article
- Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease).Annals of intensive care · 2025Article
- Intravascular Large B-Cell Lymphoma Presenting as Systemic Capillary Leak Syndrome With Immunological Phenomena: A Case Report.Case reports in hematology · 2025Article
- Immune cells play a critical role in cytokine- and endotoxin-mediated endothelial permeability.PloS one · 2025Article
- The systemic capillary leak syndrome following COVID-19 vaccine.Human vaccines & immunotherapeutics · 2024Article
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- A case of frequent of prerenal acute kidney injury attacks: importance of recognizing systemic capillary leak syndrome: a case report.The Journal of international medical research · 2024Article
- Capillary Leak Syndrome Associated with Anaplastic T Cell Lymphoma and Transcutaneous Exudation: An Unusual Presentation.Diagnostics (Basel, Switzerland) · 2024Article
- Fatal Systemic Capillary Leak Syndrome in a Patient with a COVID-19 Infection.Internal medicine (Tokyo, Japan) · 2024Article
- Intrinsic endothelial hyperresponsiveness to inflammatory mediators drives acute episodes in models of Clarkson disease.The Journal of clinical investigation · 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
- SARS-CoV-2 spike protein induces the cytokine release syndrome by stimulating T cells to produce more IL-2.Frontiers in immunology · 2024Article
- A ligand-independent Tie2-activating antibody reduces vascular leakage in models of Clarkson disease.Science advances · 2023Article
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- Capillary leak syndrome presenting as refractory ascites in a patient with lymphoma: A rare case report.The Journal of international medical research · 2023Article
- Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments.Cureus · 2023Review
- Markers of endothelial glycocalyx dysfunction in Clarkson disease.Journal of translational medicine · 2022Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
Abstract
objectivesThe Systemic Capillary Leak Syndrome (SCLS) is a rare and potentially fatal disorder resembling systemic anaphylaxis that is characterized by transient episodes of hypotensive shock and peripheral edema. The pathogenesis of SCLS is unknown, and triggers for attacks are apparent only in a minority of patients. We introduce a clinical algorithm for the diagnosis of SCLS, and we investigated potential serum biomarkers of acute SCLS episodes.
methodsWe analyzed serum cytokines in a cohort of 35 patients with an established diagnosis of SCLS and characterized the effects of SCLS sera on endothelial cell function. We investigated the cellular source(s) of CXCL10, a chemokine that was significantly elevated in both basal and acute SCLS sera, by flow cytometry.
resultsSeveral cytokines were elevated in acute SCLS sera compared to baseline or sera from healthy controls, including CXCL10, CCL2, IL-1β, IL-6, IL-8, IL-12 and TNFα. The majority of acute sera failed to activate endothelial cells as assessed by surface adhesion marker expression. Monocytes appear to be the major source of serum CXCL10, and the percentage of CXLC10+ monocytes in response to IFNγ stimulation was increased in SCLS subjects compared to controls.
conclusionsThe presence of proinflammatory cytokines in acute SCLS sera suggests that inflammation or infection may have a role in triggering episodes. The enhanced capacity of monocytes from SCLS patients to produce CXCL10 suggests a new therapeutic avenue for SCLS.
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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.