Idiopathic Systemic Capillary Leak Syndrome in Children

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.

show abstract

“…SCLS has also been reported in fourteen children during the past six years. 5, 17, 21, 25, 28, 35, 50, 52 …”

Section: Incidence Of Idiopathic Sclsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

132

155

View full text Add to dashboard Cite

show abstract

“…While allergic triggers are not typically associated with SCLS attacks, increasing evidence suggest that inflammation is often linked to leak episodes, and up to three out of four patients reported a virus-like prodromal illness preceding the leak 4. These included viral infections, such as influenza virus, respiratory syncytial virus and West Nile virus 7. Previously, a case report described the occurrence of SCLS triggered by influenza virus infection in a 40-year-old woman.…”

Section: Discussionmentioning

confidence: 99%

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

Ebdrup

Druey

Mogensen³

2018

BMJ Case Reports

View full text Add to dashboard Cite

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.

show abstract

“…101 In children diagnosed with systemic capillary leak syndrome (SCLS), hypotension, vomiting, lethargy, and abdominal pain are common. 103 As seen in adults, the key distinguishing feature between anaphylaxis and SCLS is hemoconcentration and frequent association with an antecedent viral illness. 103 In 2 studies, severe anaphylaxis in children was shown to be associated with an elevated bST value compared with children with mild and moderate anaphylaxis.…”

Section: Pediatric Considerationsmentioning

confidence: 99%

“…103 As seen in adults, the key distinguishing feature between anaphylaxis and SCLS is hemoconcentration and frequent association with an antecedent viral illness. 103 In 2 studies, severe anaphylaxis in children was shown to be associated with an elevated bST value compared with children with mild and moderate anaphylaxis. 43,104 In 1 study of children with food allergy, the highest values were seen in children with food allergy and anaphylaxis.…”

Section: Pediatric Considerationsmentioning

confidence: 99%

Idiopathic anaphylaxis yardstick

Carter

Akin

Castells

et al. 2020

Annals of Allergy, Asthma & Immunology

View full text Add to dashboard Cite

Anaphylaxis is considered idiopathic when there is no known trigger. The signs and symptoms of idiopathic anaphylaxis (IA) are identical to those of anaphylaxis because of a known cause and can include cutaneous, circulatory, respiratory, gastrointestinal, and neurologic symptoms. Idiopathic anaphylaxis can be a frustrating disease for patients and health care providers. Episodes are unpredictable, and differential diagnosis is challenging. Current anaphylaxis guidelines have little specific guidance regarding differential diagnosis and long-term management of IA. Therefore, the objective of the Idiopathic Anaphylaxis Yardstick is to use published data and the authors' combined clinical experience to provide practical recommendations for the diagnosis and management of patients with IA.

show abstract

Idiopathic Systemic Capillary Leak Syndrome in Children

Cited by 31 publications

References 21 publications

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

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

Idiopathic anaphylaxis yardstick

Contact Info

Product

Resources

About