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Rare disease
An unusual case of swelling- Clarkson’s syndrome
  1. Asif Ansari1,
  2. KC Birendra1,
  3. Mathew Marvin1,
  4. Anthony Kubat2,
  5. Timothy Fritz3
  1. 1Department of Internal Medicine, Spectrum Health, Grand Rapids, Michigan, United States
  2. 2Department of Pathology, Spectrum Health, Grand Rapids, Michigan, United States
  3. 3Department of Cardiology, Spectrum Health, Grand Rapids, Michigan, United States
  1. Correspondence to Dr Asif Ansari, ansariasif78{at}gmail.com

Summary

Clarkson’s syndrome, also called idiopathic systemic capillary leak syndrome is a rare condition characterised by vascular hyper permeability resulting in extreme intravascular volume depletion. The syndrome is unique and almost paradoxical in its presentation, with findings initially suggesting overwhelming heart failure, but in reality the extra vascular fluid represents overt capillary leak, with ultimate intravascular volume depletion, a low output state and hypovolemic shock. Previously described characteristics have classically included severe oedema and anasarca with rapid, profound shock, typically accompanied by haemoconcentration. The authors describe a patient, initially seeming benign in presentation, who rapidly progressed with confusing findings of fluid overload by examination and imaging, ultimately manifesting these findings by severe capillary leak rather than hydrostatic oedema, with ultimate hypovolaemic shock, multisystem organ failure and death. Our aim is that by describing clinical, haemodynamic and pathologic descriptors of the disease, the authors can aid in increasing physician awareness of this unusual syndrome.

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  • Competing interests None.

  • Patient consent Obtained.

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