Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson’s Disease)

Bøe, Ole Wilhelm; Svéen, Kjell; Børset, Magne; Druey, Kirk M.

doi:10.12659/ajcr.906514

Cited by 9 publications

(3 citation statements)

References 25 publications

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“…Of the thirty studies examining resuscitation and associated endotheliopathy included, eleven (37%) were on patients with trauma and haemorrhagic shock (17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27), nine (33%) on septic shock patients (28-36), and six (20%) on cardiogenic shock patients (37)(38)(39)(40)(41)(42). Two studies were case reports on systemic capillary leak syndrome (SCLS) (43,44). One study reported post-resuscitation endothelial dysfunction in acute respiratory failure (45) and another in dengue shock syndrome (46).…”

Section: Resultsmentioning

confidence: 99%

A systematic review and meta-analysis on endothelial dysfunction post-shock resuscitation

Obonyo,

Sela,

Raman

et al. 2023

Preprint

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Introduction Shock-induced endotheliopathy (SHINE), defined as a profound sympathoadrenal hyperactivation in shock states leading to endothelial activation, glycocalyx damage, and eventual compromise of end-organ perfusion, was first described in 2017. The aggressive resuscitation therapies utilised in treating shock states could potentially lead to further worsening endothelial activation and end-organ dysfunction. Objective This study aimed to systematically review the literature on resuscitation-associated and resuscitation-induced endothelial dysfunction. Methods A pre-determined structured search of literature published over an eleven-year period (1 January 2011 to 31 December 2022) was performed in two indexed databases (PubMed/MEDLINE and EMBASE) per PRISMA guidelines. Results Thirty studies met the inclusion criteria and were included in the final qualitative synthesis. Most of these studies (43%) reported on a combination of mediators released from endothelial cells and biomarkers of glycocalyx breakdown, while only 20% reported on microvascular flow changes. Only eight individual studies were included in the quantitative meta-analysis based on the comparability of the parameters assessed. Six studies measured syndecan-1, with a heterogeneity index, I2 = 75.87% (pooled effect-size; z = 2.00; mean = 0.43; 95% CI 0.01 to 0.85; p = 0.05). Thrombomodulin was measured in 4 comparable studies (I2 = 78.93%; z = 1.57; mean = 0.41; 95% CI -0.10 to 0.92; p = 0.12). Conclusion Acute resuscitation therapies administered could exacerbate endothelial dysfunction in shock states. In the included studies, syndecan-1 had the highest frequency of assessment in the post-resuscitation period and changes in concentrations showed a statistically significant effect of the resuscitation. There are inadequate data available in this area and further research and standardisation of the ideal assessment and panel of biomarkers are urgently needed.

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Section: Resultsmentioning

confidence: 99%

A systematic review and meta-analysis on endothelial dysfunction post-shock resuscitation

Obonyo,

Sela,

Raman

et al. 2023

Preprint

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“…A similar strategy has been adopted in some clinically significant cases of MGUS, with good results [ 2 ]. Elevated serum levels of Syndecan-1 (CD138) and MYOF mutation could also help in the diagnosis of this entity [ 22 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

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

Hermida,

Alvarez-Nuño,

San Miguel-Izquierdo

et al. 2024

Oncol Ther

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Background Clarkson’s disease is a very rare entity characterised by acute episodes of systemic oedema and severe hypotension associated with paraproteinaemia. Its classical treatment relies on methylxanthine combined with terbutaline. Although this prophylactic therapy reduces the mortality rate, relapses are frequent. Eighty percent of patients with Clarkson’s disease present with monoclonal gammopathy of unknown significance (MGUS). The risk of progression to multiple myeloma is 1% per year. Case Description Here, we present a 49-year-old woman who suffered multiple such episodes requiring treatment in the intensive care unit. Treatment with terbutaline and theophylline was ineffective. She was diagnosed with multiple myeloma (MM) 8 years after the first of these acute episodes. Antimyeloma treatment with bortezomib and dexamethasone was started, followed by autologous haemopoietic transplantation, with no further acute episodes since then. Conclusion Our case is, to our knowledge, unique because eradication of MM was followed by complete disappearance of acute episodes of capillary leakage. Our case report is also the first to support the use of bortezomib and dexamethasone in this setting. Furthermore, autologous peripheral blood progenitor cell transplantation consolidated the MM stringent complete remission achieving a very long progression-free survival (> 11 years) of both MM and Clarkson’s disease.

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“…However, the shedding of VEGLX components such as syndecans, versican, decorin, glypican, biglycan, mimecan and perlecan is induced (activated/induced shedding) and consequently, their circulating/soluble concentrations are increased in several systemic inflammatory diseases such as sepsis, [28][29][30][31][32][33][34][35][36] systemic lupus erythematosus, [37][38][39] rheumatoid arthritis (RA) [40][41][42] and idiopathic capillary leak syndrome. 43 Increased synthesis and secretion of these extracellular matrix components by innate immune cells such as macrophages also contribute to elevated soluble forms in the aforementioned diseases. 44,45 Further, recent studies have shown that VEGLX is damaged, and circulating concentrations of VEGLX components (e.g.…”

Section: Regulation and Mechanism Of Veglx Sheddingmentioning

confidence: 99%

Soluble vascular endothelial glycocalyx proteoglycans as potential therapeutic targets in inflammatory diseases

Kunnathattil,

Rahul,

Skaria

2023

Immunol Cell Biol

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Reducing the activity of cytokines and leukocyte extravasation is an emerging therapeutic strategy to limit tissue‐damaging inflammatory responses and restore immune homeostasis in inflammatory diseases. Proteoglycans embedded in the vascular endothelial glycocalyx, which regulate the activity of cytokines to restrict the inflammatory response in physiological conditions, are proteolytically cleaved in inflammatory diseases. Here we critically review the potential of proteolytically shed, soluble vascular endothelial glycocalyx proteoglycans to modulate pathological inflammatory responses. Soluble forms of the proteoglycans syndecan‐1, syndecan‐3 and biglycan exert beneficial anti‐inflammatory effects by the removal of chemokines, suppression of proinflammatory cytokine expression and leukocyte migration, and induction of autophagy of proinflammatory M1 macrophages. By contrast, soluble versikine and decorin enhance proinflammatory responses by increasing inflammatory cytokine synthesis and leukocyte migration. Endogenous syndecan‐2 and mimecan exert proinflammatory effects, syndecan‐4 and perlecan mediate beneficial anti‐inflammatory effects and glypican regulates Hh and Wnt signaling pathways involved in systemic inflammatory responses. Taken together, targeting the vascular endothelial glycocalyx–derived, soluble syndecan‐1, syndecan‐2, syndecan‐3, syndecan‐4, biglycan, versikine, mimecan, perlecan, glypican and decorin might be a potential therapeutic strategy to suppress overstimulated cytokine and leukocyte responses in inflammatory diseases.

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Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson’s Disease)

Cited by 9 publications

References 25 publications

A systematic review and meta-analysis on endothelial dysfunction post-shock resuscitation

A systematic review and meta-analysis on endothelial dysfunction post-shock resuscitation

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

Soluble vascular endothelial glycocalyx proteoglycans as potential therapeutic targets in inflammatory diseases

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