Capillary leak syndrome: etiologies, pathophysiology, and management.
In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a "sepsis-like" syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson's disease, engraftment syndrome, differentiation syndrome, the ovarian hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome; hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment.
Columbia University Medical Center, New York, New York, USA.
Eric
E
Siddall
Winthrop University Hospital, Mineola, New York, USA.
Minesh
M
Khatri
Columbia University Medical Center, New York, New York, USA. Electronic address: jr55@cumc.columbia.edu.
Jai
J
Radhakrishnan
D058186:Acute Kidney Injury / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D000818:Animals; D002196:Capillaries / Q000503:physiopathology*; D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D002199:Capillary Permeability; D003937:Diagnosis, Differential; D005440:Fluid Therapy / Q000009:adverse effects; D006439:Hemodynamics; D006801:Humans; D010952:Plasma Substitutes / Q000009:adverse effects / Q000627:therapeutic use*; D010996:Pleural Effusion / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D011237:Predictive Value of Tests; D012307:Risk Factors; D018805:Sepsis / Q000150:complications; D049994:Sodium Potassium Chloride Symporter Inhibitors / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome
D010952:Plasma Substitutes; D049994:Sodium Potassium Chloride Symporter Inhibitors
acute kidney injury; capillary leak syndrome; capillary permeability; cytokines