title: Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression.
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.
authors.affiliation: Intensive Care Unit, Royal Berkshire Hospital, Reading, UK.
authors.forename: James Robert
authors.initials: JR
authors.lastname: Sheehan
authors.forename: Liza
authors.initials: L
authors.lastname: Keating
authors.forename: Antoni
authors.initials: A
authors.lastname: Chan
authors.forename: Andrew
authors.initials: A
authors.lastname: Walden
mesh_terms: D019559:Capillary Leak Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D012769:Shock / Q000209:etiology*
chemical_list: D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D008775:Methylprednisolone