Capillary leak syndrome

Capillary leak syndrome

Description, Causes and Risk Factors:

Capillary leak syndrome

Capillary leak syndrome was first described by Clarkson in 1960. It is characterized by recurrent episodes of hypotension with hemoconcentration, hypoalbuminemia without albuminuria and generalized edema. These episodes are due to capillary hyperpermeability with massive extravasation of plasma containing macromolecules smaller than 200kD and sometimes as big as 900kD. Fifty-seven cases have been reported so far, patients' mean age is 46 years (ranging from 9 to 67 years). Both sexes are equally affected. Monoclonal gammopathy, generally an IgG class, is associated in 82% of cases. For some authors this gammopathy may play a role in pathogenesis, without evidence of amyloidosis. Each episode is often preceded by infection. Pathophysiology is largely misunderstood but cytokines (IL-2), leukotrienes and endothelial apoptosis have been suggested to be involved in capillary hyperpermeability. Therefore, some cases secondary to treatment by recombinant IL-2 have been reported. Each episode consists of two phases:

    The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema (sometimes responsible for rhabdomyolysis) and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.

  1. The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. Therefore, monitoring those patients is essential to timely switch to depletion treatment (including diuretics or hemofiltration).

Cappilary leak occurs in infections (sepsis, dengue shock syndrome), hereditary angioedema caused by deficiency of C1 esterase, systemic mastocytosis, chemotherapy, malignancy and hemophagocytic syndrome and these should be discarged against new cases of shock with laboratory evidence of plasma extravasation. To characterize an idiopathic form it should also be discarded Carbon monoxide poisoning, maternal status of postpartum and pustular psoriasis.

Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality. A few prophylactic treatments have been tried with variable results, principally terbutaline and theophyllin. Patients need to bemonitored for monoclonal gammopathy since it may turn into multiple myeloma.


Most patients report having a runny nose and/or other flu-like symptoms, or else gastrointestinal disorders (diarrhea or vomiting), or a general weakness or pain in their limbs, but others get no particular or consistent warning signs ahead of their episode. They subsequently develop thirst and lightheadedness and the following measurable conditions:


  • Low blood pressure (hypotension);

  • Hypoalbuminemia; and

  • Partial or generalized edema.


Capillary leak syndrome is difficult to diagnose. Diagnostic tests may include blood and urine tests and imaging studies. Doctors may diagnose sudden, periodic "attacks" of CLS by detecting concentrated blood and low serum protein in the presence of certain signs and symptoms, such as generalized body swelling, muscle aches, lightheadedness, fatigue, shortness of breath (SOB) and decreased urination — and by excluding other conditions that could cause these symptoms.


Capillary leak syndrome (CLS) frequently causes complex medical problems involving many body systems. CLS "attacks" are typically sudden, although sometimes certain warning signs or symptoms, such as body ache, flu-like symptoms, scratchy throat or dark urine, can alert you. An attack of CLS may include a marked drop in blood pressure, which if not treated promptly, can lead to multiple organ failure and death. During an episode of CLS, treatment may include:

    Fluids: Fluids are given intravenously and carefully controlled to maintain your blood pressure and to prevent damage to vital organs, such as your kidneys, heart and brain.

  • Medications: Your doctor may order medications, such as steroids, diuretics or immunoglobulin, among others. Over the long term, medications are usually prescribed to reduce the frequency and severity of future episodes.

All cases require monitoring of serum protein and calcium and adequate hidroelectrolyte, hemodynamic and temperature balance. Premisses should have a nice and humidified temperature so as to avoid hypothermia and improve skin hydration. Appropriate nutrition and control of urea, creatinine and infections are important. Sedating antihistamines can be prescribed to relieve itching and reduce anxiety. Analysis of VEGF, still being studied, might be a useful predictor for clinical outcome and its handling.

IV theophylline, terbutaline, steroids (our option, when the cause is unknown or when psoriasis is associated with acute respiratory distress syndrome or acute pulmonary edema), indomethacin, spironolactone, cyclosporine (our choice when the etiology is unstable psoriasis, plasmapheresis, prostacyclin, gingko biloba and pentastarch have been tried. Other options under study are: inhibition of apoptosis by anticaspases or antioxidants and inhibitory action on the vascular factor of endothelial growth or on pathways it mediates.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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