Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review

Marie Ange Bozzini, Gregorio P. Milani, Mario G. Bianchetti, Emilio F. Fossali, Sebastiano A.G. Lava

Research output: Contribution to journalArticlepeer-review


Approximately 500 cases of idiopathic systemic capillary leak syndrome (Clarkson syndrome) have been reported worldwide. This life-threatening condition is characterized by episodes of increase in vascular permeability with loss of fluid into the interstitium and presents with acute onset of edema, signs of tissue hypoperfusion, hemoconcentration, and low blood protein level. It has been diagnosed mainly in middle-aged adults with a monoclonal gammopathy. We performed a systematic review of the literature on Clarkson syndrome in subjects ≤ 18 years of age. We identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of Clarkson syndrome. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. A monoclonal gammopathy was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died. Conclusion: Clarkson syndrome develops not only in adulthood but also in childhood. In this age group, this condition is not linked to a monoclonal gammopathy.(Table presented.)

Original languageEnglish
Pages (from-to)1149–1154
Number of pages6
JournalEuropean Journal of Pediatrics
Issue number8
Publication statusPublished - 2018


  • Acute kidney injury
  • Capillary leak syndrome
  • Capillary permeability
  • Compartment syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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