Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease)
|Zhihui Xie1*, Eunice Chan1, Yuzhi Yin1, Chandra C. Ghosh2, Laura Wisch1, Celeste Nelson1, Michael Young3, Samir M. Parikh2
and Kirk M. Druey1
|1 Laboratory of Allergic Diseases, NIAID/NIH, Bethesda, MD, USA|
|2 Department of Medicine, Division of Nephrology and Center for Vascular Biology Research, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA|
|3 Clinical Research Directorate/CMRP, Leidos Biomedical Research Inc., Frederick, National Laboratory for Cancer Research, Frederick, MD, USA|
|Corresponding Author :||Zhihui Xie
10 Center Drive, Room 11N242 Bethesda
MD 20892, USA
E-mail: [email protected]
|Received February 26, 2014; Accepted April 28, 2014; Published April 30, 2014|
|Citation: Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, et al. (2014) Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). J Clin Cell Immunol 5:213. doi: 10.4172/2155-9899.1000213|
|Copyright: © 2014 Xie Z, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.|
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Objectives: The 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.
Methods: We 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.
Results: Several 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.
Conclusions: The 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.