Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

Sheehan, James Robert; Keating, Liza; Chan, Antoni; Walden, Andrew

doi:10.1136/bcr-2013-009048

Cited by 7 publications

(2 citation statements)

References 12 publications

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“…It is thought that in some patients with SCLS, inflammatory cytokines may play a role in inducing and/or maintaining microvascular permeability. The increased serum IL-6 and TNF-α levels, 11 , 12 the improved clinical condition obtained in some patients with TNF inhibitors during acute episodes, 11 and the use of IVIG both in the acute phase and as disease prophylaxis, 5 , 13 , 14 support this view. 11 , 12 A similar conclusion can be reached for the syndrome described here, in which the presence of high fever, neutrophilic leukocytosis and high serum CRP levels, besides the prompt response to steroids and IVIG, supported autoinflammation as the underlying pathogenic mechanism.…”

Section: Discussionmentioning

confidence: 84%

Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins

Prete

Urso

Fatone³

et al. 2016

Medicine

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This report describes the onset of systemic capillary leak (SCL)-like syndrome in a 30-year-old woman with antiphospholipids syndrome (APS) during puerperium.Twelve hours after a cesarean section, she presented a sudden fever and abdominal pains followed by dyspnea, severe edema of the limbs and pelvis.Computer tomography shows congestion of interstitial pulmonary parenchyma, pericardial and pleural effusion, edema of intestinal wall and of perivisceral adipose tissue, and periportal lymphedema. Laboratory tests showed neutrophilic leukocytosis, hypoalbuminemia, and an increase of erythrocyte sedimentation rate and C-reactive protein. Because fever and raised inflammation parameters are not observed in idiopathic capillary leak syndrome (SCLS; Clarkson disease), a diagnosis of SCL-like syndrome was made.Albumin solution, high-dose methylprednisolone and intravenous immunoglobulins (IVIG) infusion were administered with a rapid improvement of her clinical condition.The prompt treatment with steroids and IVIG likely prevented the life-threatening shock syndrome that can occur in SCLS, with acute hypotensive attacks, and severe limbs edema requiring fasciotomy.All clinical and laboratory findings supported autoinflammation as the underlying pathogenic mechanism of the syndrome. The data indicate that SCL-like syndrome can be considered a novel clinical syndrome, which can complicate APS.

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Section: Discussionmentioning

confidence: 84%

Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins

Prete

Urso

Fatone³

et al. 2016

Medicine

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“…Currently, there is no specific therapy for CLS other than supportive measures, including mechanical ventilation, vasopressors, and careful fluid administration. Systemic steroids and IV immunoglobulines have been proven to be effective in several case reports [9,26]. Our patient did not properly respond to systemic steroids and immunoglobulins, and we used CPFA in the attempt to interrupt the cytokine storm that, we believe, was sustaining her critical condition.…”

Section: Discussionmentioning

confidence: 86%

Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report

Maso¹,

Cozzi²,

Gerini³

et al. 2020

Blood Purif

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AbstarctCoupled plasma filtration adsorption (CPFA) is an extracorporeal supportive therapy based on nonspecific adsorption of pro-and anti-inflammatory mediators combined with continuous renal replacement therapy. The main field of CPFA application is septic shock, and there are limited data about its efficacy in the treatment of other acute conditions characterized by a dysregulation in immune homeostasis. Capillary leak syndrome (CLS) defines a life-threatening condition sustained by hypercytokinemia and characterized by abrupt onset of increased capillary permeability leading to severe generalized edema and hypovolemic shock refractory to fluid administration. Therapy for CLS is not specific and, at present time, it consists in the use of steroids or intravenous immunoglobulins. We present the case of a 34-yearold woman who developed CLS superimposed to acute generalized exanthematous pustulosis after initiating therapy with hydroxychloroquine for undifferentiated connective tissue disease. CLS did not respond to steroids and intravenous immunoglobulins, while it was successfully treated with CPFA. This observation supports the possible role of CPFA in restoring a proper immunologic homeostasis not only in sepsis but also in other devastating conditions sustained by hypercytokinemia.

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Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

145

172

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In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

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Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

Cited by 7 publications

References 12 publications

Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins

Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins

Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report

Idiopathic systemic capillary leak syndrome (Clarkson disease)

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