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

BMJ Case Rep. 2013 Apr 9:2013:bcr2013009048. doi: 10.1136/bcr-2013-009048.

Abstract

A female patient in her 60s presented with a history of malaise, chills, headache and vomiting. She was in shock on presentation with a high haematocrit and a low albumin with evidence of rhabdomyolysis. Severe limb and truncal oedema developed with worsening hypotension leading to intensive care unit admission for multiple organ support. Extensive radiological, microbiological and immunological work up was negative with the exception of a monoclonal gammopathy. A review of patient investigations led to a diagnosis of Clarkson's disease. Treatment with high-dose methylprednisolone and intravenous immunoglobulins led to a rapid decline in the creatine kinase (CK) level and vasopressor requirements. The patient was discharged home on long-term terbutaline and has made a good recovery.

Publication types

  • Case Reports

MeSH terms

  • Capillary Leak Syndrome / complications*
  • Capillary Leak Syndrome / diagnosis*
  • Capillary Leak Syndrome / drug therapy
  • Diagnosis, Differential
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Shock / etiology*

Substances

  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Methylprednisolone