The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission

Chambrun, Marc Pineton de; Luyt, Charles‐Édouard; Beloncle, François; Gousseff, Marie; Mauhin, Wladimir; Argaud, Laurent; Ledochowski, Stanislas; Moreau, Anne‐Sophie; Sonneville, Romain; Verdière, Bruno; Merceron, Sybille; Zappella, Nathalie; Landais, Mickaël; Contou, Damien; Demoule, Alexandre; Paulus, S.; Souweine, Bertrand; Lecomte, Bernard; Vieillard‐Baron, Antoine; Terzi, Nicolas; Azoulay, Élie; Friolet, Raymond; Puidupin, M.; Devaquet, Jérôme; Mazou, Jean-Marc; Fédun, Yannick; Mira, Jean‐Paul; Raphalen, Jean-Herlé; Combes, Alain; Amoura, Zahir

doi:10.1097/ccm.0000000000002496

Cited by 62 publications

(58 citation statements)

References 27 publications

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“…There may be a benefit of immunomodulatory therapy in patients with MIS-C, severe disease, and evidence of cytokine storm syndrome and/or those with cardiac involvement. Due to recent emergence of MIS-C, no randomized trials or comparative effectiveness studies have evaluated treatment strategies, but the benefits of immunomodulatory therapy are well established in KD [ 39 ], and they are often used for the treatment of infective myocarditis [ 46 – 48 ] and other systemic inflammatory diseases [ 49 , 50 ]. Therefore, based on the experience in similar conditions, it appears reasonable to suggest an immunomodulation approach based on intravenous immunoglobulins (IVIGs).…”

Section: Managementmentioning

confidence: 99%

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach

et al. 2020

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Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2-6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease. Here, we present a narrative review of cases reported in literature and we discuss the clinical acute and follow-up management of these patients. Patients with SARS-Cov-2-associated multisystem inflammatory syndrome frequently presented with persistent fever, gastrointestinal symptoms, polymorphic rash, conjunctivitis, and mucosal changes. Elevated inflammatory markers and evidence of cytokine storm were frequently observed. A subset of these patients also presented with hypotension and shock (20-100%) from either acute myocardial dysfunction or systemic hyperinflammation/vasodilation. Coronary artery dilation or aneurysms have been described in 6-24%, and arrhythmias in 7-60%. Cardiac support, immunomodulation, and anticoagulation are the key aspects for the management of the acute phase. Long-term structured follow-up of these patients is required due to the unclear prognosis and risk of progression of cardiac manifestations. Conclusion: Multisystem inflammatory syndrome is a novel syndrome related to SARS-CoV-2 infection. Evidence is still scarce but rapidly emerging in the literature. Cardiac manifestations are frequent, including myocardial and coronary involvement, and need to be carefully identified and monitored over time.

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Section: Managementmentioning

confidence: 99%

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach

et al. 2020

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“…A recent multicentre study of patients with SCLS admitted to the ICU demonstrated that therapy of acute flares with high-volume intravenous fluids was independently associated with poorer outcomes 5. Although a beneficial effect of IVIG (1–2 g/kg) during acute leaks has been suggested based on the study of a few patients,13 overall, its efficacy in the acute setting has not been established 5. In contrast, prophylactic administration of IVIG on a monthly basis clearly reduces the severity and frequency of episodes in most patients 4 14.…”

Section: Discussionmentioning

confidence: 99%

Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection

Ebdrup

Druey

Mogensen³

2018

BMJ Case Reports

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Systemic capillary leak syndrome (SCLS), also known as Clarkson syndrome, is a rare disease with potential fatal outcome. The clinical picture involves leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension. The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock. We present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome. The severe episode of capillary leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode. Here we describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.

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“…Other possible triggers of CLS include idiopathic, infection, ischemia-reperfusion injury, and metformin. Idiopathic CLS, as known as Clarkson syndrome, [ 6 , 16 ] is a rare syndrome with less than 500 cases being reported so far. The typical presentation of Clarkson syndrome is recurrent episodes of increased capillary permeability with viral-like prodrome in the context of monoclonal gammopathy.…”

Section: Discussionmentioning

confidence: 99%

“…Acute limb compartment syndrome is a good example that is accompanied by CLS and is a surgical emergency. [ 5 , 6 ] Moreover, acute compartment syndrome accompanied by CLS shows atypical presentation, which make diagnosis challenging. Here, we report a case of severe CLS following a metformin and dipeptidyl peptidase-4 (DPP-4) inhibitors overdose, which resulted in multiple limb compartment syndrome.…”

Section: Introductionmentioning

confidence: 99%

Multiple limb compartment syndrome as a manifestation of capillary leak syndrome secondary to metformin and dipeptidyl peptidase IV inhibitor overdose

Kasugai

Kosuke²,

Jingushi

et al. 2020

Medicine

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Rationale: Capillary leak syndrome is a condition that increases systemic capillary permeability and causes characteristic manifestations such as recurrent hypovolemia, systemic edema, and hemoconcentration. Acute limb compartment syndrome is a possible complication of severe capillary leak syndrome. However, timely diagnosis and prompt treatment are challenging because of atypical presentation. Patient concerns: An 18-year-old woman with a history of clinical depression was admitted to our intensive care unit (ICU) because of metformin and vildagliptin overdose. She developed marked vasodilatory shock with recurrent severe hypovolemia and disseminated intravascular coagulation. After urgent hemodialysis and plasma exchange, she started to stabilize hemodynamically. However, her limbs became stone-hard with massive edema. Her serum creatinine kinase level increased to an extremely high level. Diagnosis: Extremities were distended, and her skin developed pallor with blistering. Intramuscular pressure in both forearms and lower legs was significantly elevated. Interventions: Decompressive fasciotomy was performed. Hemodialysis was continued because of rhabdomyolyses-induced acute kidney injury. Outcomes: The patient was finally able to walk by herself at the time of hospital discharge on day 109. Lessons: The possibility of acute compartment syndrome should be considered in patients with marked capillary leakage, especially after aggressive fluid resuscitation. It is important to be aware of the compartment syndrome in an ICU setting because communication barriers often mask typical symptoms and make diagnosis difficult.

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The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission

Cited by 62 publications

References 27 publications

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach

Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection

Multiple limb compartment syndrome as a manifestation of capillary leak syndrome secondary to metformin and dipeptidyl peptidase IV inhibitor overdose

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