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

@article{PinetondeChambrun2017TheCP,
  title={The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission},
  author={Marc Pineton de Chambrun and Charles-Edouard Luyt and François Beloncle and Marie Gousseff and Wladimir Mauhin and Laurent Argaud and Stanislas Ledochowski and Anne Sophie Moreau and Romain Sonneville and Bruno Verdi{\`e}re and Sybille Merceron and Nathalie Zappella and Mickael Landais and Damien Contou and Alexandre Demoule and Sylvie Paulus and Bertrand Souweine and Bernard Lecomte and Antoine Vieillard-Baron and Nicolas Terzi and {\'E}lie Azoulay and Raymond Friolet and Marc Puidupin and J{\'e}r{\^o}me Devaquet and J M Mazou and Yannick F{\'e}dun and Jean Paul Mira and Jean-Herl{\'e} Raphalen and Alain Combes and Zahir Amoura},
  journal={Critical Care Medicine},
  year={2017},
  volume={45},
  pages={1216–1223}
}
Objective: Systemic capillary-leak syndrome is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission. This study was undertaken to describe the clinical pictures and ICU management of severe systemic capillary-leak syndrome episodes. Design, Setting, Patients: This multicenter retrospective analysis concerned patients entered in the European Clarkson’s disease (Eur… 
Severe Exacerbations of Systemic Capillary Leak Syndrome After COVID-19 Vaccination: A Case Series
TLDR
3 patients who had severe flares of SCLS immediately after receiving standard doses of the COVID-19 vaccines that have emergency use authorization from the U.S. Food and Drug Administration are described to alert clinicians to the possibility of SclS-like events immediately after CO VID-19 vaccination.
Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more
TLDR
40 years’ experience in treating shock in ISCLS patients is summarized to derive a therapeutic algorithm and Complications listed by frequency in patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema and signs of compartment syndrome.
Handling shock in idiopathic systemic capillary leak syndrome (Clarkson's disease) less is more: reply
TLDR
40 years' experience in treating shock in ISCLS patients is summarized to derive a therapeutic algorithm and Complications listed by frequency in patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema and signs of compartment syndrome.
Capillary leak syndrome with tamponade
TLDR
The ongoing need for large-volume fluid resuscitation with multiple sites of third-spaced fluid and haemoconcentration prompted the consideration of systemic capillary leak syndrome (CLS), or Clarkson’s syndrome, a rare syndrome characterised by plasma and plasma proteins leaking from capillaries.
Systemic capillary leak syndrome complicated by laryngeal edema after severe acute respiratory syndrome coronavirus 2 vaccination
TLDR
A patient who had an exacerbation of SCLS requiring intensive care after receiving a second dose of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine is described.
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review
TLDR
An up-to-date, evidence-based review of the understanding of Idiopathic systemic capillary leak syndrome and its management in the light of currently available evidence is provided.
A Rare Case of Idiopathic Capillary Leak Syndrome
TLDR
A 35-year-old male with a past medical history of asthma and gastroesophageal reflux disease who initially presented to the hospital for the treatment of chronic demyelinating inflammatory neuropathy is presented, requiring plasmapheresis and steroid therapy for ICLS.
Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection
TLDR
A case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome, and the severe episode of capillary leak was potentially triggered by influenza virus infection.
Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more—comment
TLDR
A very accurate and comprehensive picture of idiopathic systemic capillary leak syndrome (ISCLS) acute episodes is presented and a clear and valid diagnostic-therapeutic algorithm underlines correctly the importance of the monoclonal component in the diagnosis of ISCLS is offered.
...
...

References

SHOWING 1-10 OF 30 REFERENCES
The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry
TLDR
Clinical experience with these 28 patients with SCLS suggests that prophylactic treatment with β(2)-agonists or intravenous immunoglobulin may reduce the frequency and severity of attacks and may improve survival.
Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience.
TLDR
A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empirical therapies and the rate of progression to multiple myeloma appears to be comparable to that of monoclonal gammopathy of undetermined significance.
Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.
Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report
TLDR
The case of a patient with idiopathic systemic capillary leak syndrome who developed an unexpected and potentially fatal abdominal and four-limb compartment syndrome is reported, which is the first report of this kind in the literature.
[Idiopathic capillary leak syndrome].
Narrative Review: The Systemic Capillary Leak Syndrome
TLDR
Clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension, and patients who survive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%.
Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years.
TLDR
An initial diagnosis of sepsis was made, but the lack of fever and absence of a demonstrable organism or source led to a revision of diagnosis to SLCS, and this article reviews these cases.
Systemic capillary leak syndrome resulting in compartment syndrome and the requirement for a surgical airway
TLDR
A case of systemic capillary leak syndrome associated with a monoclonal band on plasma electrophoresis, precipitated by ischaemic pain in the left lower limb, associated with polycythaemia, renal failure and hypovolaemic shock is reported.
The Systemic Capillary Leak Syndrome
...
...