Markers of endothelial glycocalyx dysfunction in Clarkson disease

被引:4
作者
Xie, Zhihui [1 ]
Borset, Magne [2 ,3 ]
Sveen, Kjell [2 ]
Boe, Ole Wilhelm [2 ]
Chan, Eunice C. [1 ]
Lack, Justin B. [4 ]
Hornick, Katherine M. [4 ]
Verlicchi, Franco [5 ]
Eisch, A. Robin [1 ]
Melchio, Remo [6 ]
Dudek, Arkadiusz Z. [7 ]
Druey, Kirk M. [1 ]
机构
[1] NIAID, Lung & Vasc Inflammat Sect, Lab Allerg Dis, NIH, 10 Ctr Dr,Room 11N238A, Bethesda, MD 20892 USA
[2] Norwegian Univ Sci & Technol, Dept Clin & Mol Med, Trondheim, Norway
[3] St Olavs Univ, Dept Immunol & Transfus Med, Trondheim, Norway
[4] NIAID, NIAID Collaborat Bioinformat Resource, NIH, Hlth, Bethesda, MD 20892 USA
[5] Transfus Serv Ravenna, Romagna Hlth Unit, Transfus Med Faenza Lugo, Ravenna, Italy
[6] Santa Croce & Carle Hosp, Dept Internal Med, Via Michele Coppino 26, Cuneo, Italy
[7] HealthPartners Neurosci Ctr, St Paul, MN USA
基金
美国国家卫生研究院;
关键词
Capillary leak; Endothelium; Glyocalcyx; CAPILLARY; ICU;
D O I
10.1186/s12967-022-03587-1
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
Background Clarkson disease (monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome, ISCLS) is a rare idiopathic condition marked by transient, relapsing-remitting episodes of systemic microvascular hyper-permeability, which liberates plasma fluid and macromolecules into the peripheral tissues. This pathology manifests clinically as the abrupt onset of hypotensive shock, hemoconcentration, and hypoalbuminemia. Methods We analysed endothelial glycocalyx (eGCX)-related markers in plasma from patients with ISCLS during acute disease flares and convalescence by ELISA and comprehensive proteomic profiling. We evaluated eGCX-related components and gene expression in cultured endothelial cells using RNA-sequencing, real-time PCR, and fluorescence staining. Results Serum levels of eGCX-related core components including hyaluronic acid (HA) and the core proteoglycan soluble syndecan-1 (sCD138) were elevated at baseline and during acute ISCLS flares. Serial measurements demonstrated that sCD138 levels peaked during the recovery (post-leak) phase of the illness. Proteomic analysis of matched acute and convalescent ISCLS plasma revealed increased abundance of eGCX-related proteins, including glypicans, thrombospondin-1 (TSP-1), and eGCX-degrading enzymes in acute compared to remission plasma. Abundance of endothelial cell damage markers did not differ in acute and baseline plasma. Expression of several eGCX-related genes and surface carbohydrate content in endothelial cells from patients with ISCLS did not differ significantly from that observed in healthy control cells. Conclusions eGCX dysfunction, but not endothelial injury, may contribute to clinical symptoms of acute ISCLS. Serum levels of of eGCX components including sCD138 may be measured during acute episodes of ISCLS to monitor clinical status and therapeutic responses.
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页数:10
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