Clarkson’s Disease Episode or Secondary Systemic Capillary Leak-Syndrome

Chambrun, Marc Pineton de; Constantin, Jean Michel; Mathian, Alexis; Quemeneur, Cyril; Lepère, Victoria; Luyt, Charles Edouard; Amoura, Zahir

doi:10.1016/j.chest.2020.07.084

Cited by 6 publications

(6 citation statements)

References 5 publications

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“…Information from our studies complements the findings of several recent case reports of severe SCLS attacks associated with mild-to-moderate SARS-CoV-2 infection ( 9 – 12 ). In each of the cases in those studies, patients exhibited all the hallmarks of severe SCLS exacerbations after experiencing mild-to-moderate symptoms of COVID-19; 2 of 3 patients died from SCLS-related complications.…”

Section: Discussionsupporting

confidence: 81%

Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease

Cheung¹,

Eisch²,

Maleque³

et al. 2021

Emerg. Infect. Dis.

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We report 2 fatal exacerbations of systemic capillary leak syndrome (SCLS), also known as Clarkson disease, associated with coronavirus disease (COVID-19) in the United States. One patient carried an established diagnosis of SCLS and the other sought treatment for new-onset hypotensive shock, hemoconcentration, and anasarca, classic symptoms indicative of an SCLS flare. Both patients had only mild-to-moderate symptoms of COVID-19. This clinical picture suggests that these patients succumbed to complications of SCLS induced by infection with severe acute respiratory syndrome coronavirus 2. Persons with known or suspected SCLS may be at increased risk for developing a disease flare in the setting of mild-to-moderate COVID-19 infection.

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Section: Discussionsupporting

confidence: 81%

Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease

Cheung¹,

Eisch²,

Maleque³

et al. 2021

Emerg. Infect. Dis.

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“…There is current thoughts that in COVID-19 cytokine storm syndrome IL-6 plays a central role [ 19 ]. In SCLS, there appears to be low IL-6 and CRP initially which appears to separate these two diseases [ 15 ]. Whether our therapeutic approach—IVIG, steroids, remdesivir, CRRT, and heparin—was efficacious is unknown.…”

Section: Discussionmentioning

confidence: 99%

“…The case described here adds to the existing body of knowledge surrounding COVID-19-associated SCLS and the range of therapies administered. There are three different etiologies of COVID-19-associated SCLS in the literature to date: (1) new onset [ 13 , 14 ], (2) exacerbations of preexisting SCLS (de [ 15 ]), and (3) vaccine-induced SCLS [ 16 , 17 ].…”

Section: Introductionmentioning

confidence: 99%

New-Onset Systemic Capillary Leak Syndrome in an Adult Patient with COVID-19

Knox

Lee

Leither

et al. 2021

Case Reports in Critical Care

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Systemic capillary leak syndrome (SCLS) is a rare disorder manifesting as shock, hemoconcentration, and hypoalbuminemia, which may be either idiopathic or secondary to an underlying disease process. We report a case of an adult with COVID-19 infection associated with new onset SCLS. Our case joins only two other cases of new SCLS associated with COVID-19 infection reported in the literature. The clinical and laboratory findings in this case are distinct from findings seen in COVID-19 cytokine storm syndrome. Whether our therapeutic approach was efficacious is unknown. Further research into causes and therapies for these syndromes is urgently indicated.

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“…Diagnosis of idiopathic SCLS is based on the acute episodic attacks of systemic capillary hyperpermeability and by the presence (in >90% of cases) of monoclonal gammopathy [10], whereas secondary SCLS is caused by malignant hematological diseases, viral infections, and treatments such as chemotherapies and therapeutic growth factors [13]. It is important to differentiate between idiopathic and secondary SLCS because of different therapeutic implications [14]. While no specific drug has proven effective during the acute phase for both idiopathic and secondary SCLS [13], proper treatment for the underlying cause of secondary SCLS is important.…”

Section: Introductionmentioning

confidence: 99%

A Disproportionality Analysis for Association of Systemic Capillary Leak Syndrome with COVID-19 Vaccination Using the World Health Organization Pharmacovigilance Database

2022

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Systemic capillary leak syndrome (SCLS) is a rare and potentially life-threatening disorder characterized by reversible plasma extravasation and vascular collapse. This study aimed to investigate the association between different types of COVID-19 vaccine and SCLS in a real-world setting. We used individual case safety reports of SCLS after COVID-19 vaccination from the WHO pharmacovigilance database, VigiBase. A disproportionality analysis of ChAdOx1 nCoV-19 and mRNA-based vaccines was performed. The information component (IC) and reporting odds ratio (ROR) were calculated from the entire database and viral vaccines data subset. A positive 95% lower end of the IC (IC025) value (>0) using Bayesian neural network analysis and lower end of the ROR 95% confidence interval (ROR025) ≥1 were defined as the ADR signal detection threshold. A total of 101 (0.004%) events of SCLS were identified. A significant potential signal of disproportionality of SCLS was noted in ChAdOx1 nCoV-19 when applied as the denominator for entire database (IC025 = 0.24, ROR025 = 1.23) and all viral vaccines (IC025 = 0.41, ROR025 = 1.59). No significant potential signal was noted for two mRNA-based vaccines as denominators for the entire database (IC025 = −0.49, ROR025 = 0.71) and all viral vaccines (IC025 = −0.32, ROR025 = 0.77). Contrary to ChAdOx1 nCoV-1, no safety signal for developing SCLS was identified for mRNA-based vaccines.

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Clarkson’s Disease Episode or Secondary Systemic Capillary Leak-Syndrome

Cited by 6 publications

References 5 publications

Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease

Fatal Exacerbations of Systemic Capillary Leak Syndrome Complicating Coronavirus Disease

New-Onset Systemic Capillary Leak Syndrome in an Adult Patient with COVID-19

A Disproportionality Analysis for Association of Systemic Capillary Leak Syndrome with COVID-19 Vaccination Using the World Health Organization Pharmacovigilance Database

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