Capillary leak syndrome

Capillary leak syndrome
Classification and external resources
DiseasesDB	34090
MeSH	D019559
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Capillary leak syndrome (also known as systemic capillary leak syndrome, SCLS, or Clarkson’s disease) is an extremely 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), , 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.

The syndrome was first described by B. Clarkson in 1960, 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.

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:

An episode of SCLS usually consists of two distinct phases:

The initial stage is the capillary leak phase, lasting from 1 to 3 days, during which up to 70% of total plasma volume may invade cavities in the trunk and extremities. 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.

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