ArticleAnnals of internal medicine. Clinical cases2022
Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins.
Article in Annals of internal medicine. Clinical cases, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
What it found
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
10 citing papers in PubMed, 14 citations in OpenAlex.
- Precision Monitoring in a Sepsis Mimic: A Case Report of Idiopathic Capillary Leak Syndrome in the Surgical ICU.Healthcare (Basel, Switzerland) · 2026Article
- Case Report: Rare co-occurrence of NMOSD and capillary leak syndrome treated with satralizumab.Frontiers in immunology · 2026Review
- [Systemic capillary leak syndrome: A case of severe presentation and treatment response].Anales del sistema sanitario de Navarra · 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
- Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease).Annals of intensive care · 2025Article
- Systemic capillary leak syndrome.Nature reviews. Disease primers · 2024Review
- 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
- A ligand-independent Tie2-activating antibody reduces vascular leakage in models of Clarkson disease.Science advances · 2023Article
- Autologous hematopoietic stem cell transplantation in Clarkson disease.The journal of allergy and clinical immunology. In practice · 2023Article
Corrections and comments
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Authors and funding
6 authors at 5 institutions in 2 countries.
Funding
Abstract
Monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome (ISCLS, Clarkson disease) is a rare disorder defined by transient but recurrent bouts of hypotensive shock and anasarca resulting from plasma extravasation. Although prophylactic treatment with high-dose intravenous immunoglobulins (IVIG, 1-2 g/kg/mo) prevents most disease flares, its utility for acute episodes of ISCLS is unclear. Here, we report the results of a retrospective study of subjects with acute ISCLS treated at or near the onset of symptoms with IVIG. We found that administration of IVIG with minimal additional intravenous fluids was safe and associated with rapid clinical improvement. IVIG given close to the onset of ISCLS-related symptoms is associated with a favorable outcome.
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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.