| Capillary leak syndrome | |
|---|---|
| Classification and external resources | |
| DiseasesDB | 34090 |
| MeSH | D019559 |
Capillary leak syndrome (usually Systemic Capillary Leak Syndrome, SCLS or Clarkson’s Disease) is a rare medical condition characterized by self-reversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for a leakage of fluid from the circulatory system to the interstitial space, resulting in a dangerous hypotension (low blood pressure), hemoconcentration, and hypoalbuminemia. It is a life-threatening illness because each episode has the potential to cause damage to, or the failure of, vital organs due to limited perfusion. It is often misdiagnosed as polycythemia, polycythemia vera or sepsis.
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The syndrome was first described by B. Clarkson in 1960,[1] after whom it was later informally named. Beyond numerous case reports published since then, two comprehensive reviews of clinical and research experience were published in 2010.[2][3]
Most patients report having a runny nose and/or other flu-like symptoms, or else gastro-intestinal 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:[2][3]
An episode of SCLS usually consists of two distinct phases:[2][3]
The initial stage is the capillary leak phase, lasting from 1 to 4 days. The most common clinical features are fatigue; lightheadedness up to and including syncope (fainting); limb, abdominal or generalized pain; facial or other edema; dyspnea; and hypotension that results in circulatory shock and potentially in cardiopulmonary collapse and other organ distress or damage. Acute renal failure is a risk due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.
The loss of fluid out of the capillaries has similar effects on the circulation as dehydration, slowing both the flow of oxygen delivered to tissues and organs as well as the output of urine. Urgent medical attention in this phase consists of fluid resuscitation efforts, mainly the intravenous administration of saline solution plus hetastarch or albumin and colloids (to increase the remaining blood flow to vital organs like the kidneys), as well as glucocorticoids (steroids like methylprednisolone, to reduce or stop the capillary leak). However, it is important to avoid overly aggressive intravenous fluid administration during this leak phase, because it may cause massive swelling of the extremities and thus serious collateral damage because of induced compartment syndromes.
The second stage features the reabsorption of the initially extravasated fluid and usually lasts from 1 to 3 days. Intravascular fluid overload leads to polyuria and can cause flash pulmonary edema with possibly fatal consequences. The severity of the problem depends on to the quantity of fluid supplied in the initial phase, the damage that may have been sustained by the kidneys, and the promptness with which diuretics are administered to help the patient discharge the accumulated fluids quickly.
The prevention of episodes of SCLS has involved two approaches. The first has long been identified with the Mayo Clinic and it recommends treatment with beta agonists such as theophylline, terbutaline and montelukast sodium.[4] The second, more recent approach pioneered in France involves monthly intravenous infusions of intravenous immunoglobulin (IVIG), and the case-report evidence is encouraging.[5][6][7]
A recent review of clinical experience with 28 European SCLS patients suggests that either prophylactic treatment may reduce the frequency and severity of attacks and may improve survival.[8]
In the Mayo Clinic’s experience, the median survival of 25 patients that were followed over 30 years (counting only SCLS-related deaths) was approximately 15 years, and their 5-year survival rate was 76%.[2][3] In European experience, the 5-year post-diagnosis survival rate was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not.[8] However, better identification and management of this condition appears to be resulting in lower mortality and improving survival and quality-of-life results as of late.
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