A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.
References
- Eo TS, Chun KJ, Hong SJ, et al. JI Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. J Allergy Clin Immunol Pract 2018; 6(2): 609–618. doi: 10.1016/j.jaip.2017.07.021.Epub 2017 Sep 19.
- Xie Z, Ghosh CC, Patel R, et al. Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood 2012; 119(18):4321–4332. doi:10.1182/blood-2011-08-375816.
- Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol 2017; 140(3):663–670. doi:10.1016/j.jaci.2016.10.042.
- Xie Z, Nagarajan V, Sturdevant DE, et al. Genome-wide SNP analysis of the Systemic Capillary Leak Syndrome (Clarkson disease). Rare Dis 2013; 1(1): e27445. doi:10.4161/rdis.27445.
- Gousseff M, Arnaud L, Lambert M, et al. Capillary Leak Syndrome Registry. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med 2011; 154(7):464–71. doi: 10.7326/0003-4819-154-7-201104050-00004.
- Ebdrup L, Druey KM, Druey K, et al. Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection [published correction appears in BMJ Case Rep 2018. Druey K [corrected to Druey KM]]. BMJ Case Rep 2018; bcr2018226108.Published 2018 Aug 29. doi:10.1136/bcr-2018-226108.
- Pineton de Chambrun M, Luyt CE, Beloncle F, et al. The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. Crit Care Med 2017; 45(7):1216–1223. doi: 10.1097/CCM.0000000000002496.
- Lambert M, Launay D, Hachulla E, et al. High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome. Crit Care Med 2008; 36(7): 2184–7. doi: 10.1097/CCM.0b013e31817d7c71.
- Pineton de Chambrun M, Gousseff M, Mauhin W, et al. Intravenous immunoglobulins improve survival in monoclonal gammopathy associated systemic capillary-leak syndrome. Am J Med 2017; 130:e19–1219.e27. 10.1016/j.amjmed.2017.05.023.
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