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Revision as of 19:19, 28 January 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Synonyms and Keywords: Systemic capillary leak syndrome; SCLS; Clarkson's disease.
Overview
Capillary leak syndrome 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.
Historical Perspective
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]
Natural History
The episode usually consists of two phases
1. The capillary leak phase (1-4 days) The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.
2. Recruitment of the interstitial fluid 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. It's necessary to monitor the patient in order to switch to depletion treatment with diuretics or hemofiltration.
Prognosis
Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
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.[4] 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.
Diagnosis
Symptoms
Generalized swelling may be present.
Physical Examination
Vitals
Skin
Laboratory Studies
- Hemoconcentration
- Hypoalbuminemia without albuminuria
Treatment
A few prophylactic treatments have been tried with variable results, principally terbutaline and theophylline.
An episode of SCLS usually consists of two distinct phases:
The capillary leak phase
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.
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 recruitment phase
The second stage features the reabsorption of the initially extravasated fluid and it usually lasts just as long as the leak phase. 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.[5] The second, more recent approach pioneered in France involves monthly intravenous infusions of intravenous immunoglobulin (IVIG), and the growing case-report evidence is encouraging.[6][7][8][9]
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.[4]
Case Reports
Sources
- Dorlands Medical Dictionary 30th Ed.
- Cancer.gov
- Orphanet (2003)
References
- ↑ Clarkson, Bayard; Thompson, David; Horwith, Melvin; Luckey, E.Hugh (1960). "Cyclical edema and shock due to increased capillary permeability". The American Journal of Medicine. 29 (2): 193–216. doi:10.1016/0002-9343(60)90018-8. PMID 13693909.
- ↑ 2.0 2.1 Druey, Kirk M.; Greipp, Philip R. (2010). "Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome". Annals of Internal Medicine. 153 (2): 90–8. doi:10.1059/0003-4819-153-2-201007200-00005. PMC 3017349. PMID 20643990.
- ↑ 3.0 3.1 Kapoor, Prashant; Greipp, Patricia T.; Schaefer, Eric W.; Mandrekar, Sumithra J.; Kamal, Arif H.; Gonzalez-Paz, Natalia C.; Kumar, Shaji; Greipp, Philip R. (2010). "Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience". Mayo Clinic Proceedings. 85 (10): 905–12. doi:10.4065/mcp.2010.0159. PMC 2947962. PMID 20634497.
- ↑ 4.0 4.1 Gousseff, Marie; Arnaud, Laurent; Lambert, Marc; Hot, Arnaud; Hamidou, Mohamed; Duhaut, Pierre; Papo, Thomas; Soubrier, Martin; Ruivard, Marc (2011). "The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry". Annals of Internal Medicine. 154 (7): 464–71. doi:10.1059/0003-4819-154-7-201104050-00004. PMID 21464348.
- ↑ Droder, RM; Kyle, RA; Greipp, PR (1992). "Control of systemic capillary leak syndrome with aminophylline and terbutaline". The American Journal of Medicine. 92 (5): 523–6. doi:10.1016/0002-9343(92)90749-2. PMID 1580299.
- ↑ Lambert, Marc; Launay, David; Hachulla, Eric; Morell-Dubois, Sandrine; Soland, Vincent; Queyrel, Viviane; Fourrier, François; Hatron, Pierre-Yves (2008). "High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome". Critical Care Medicine. 36 (7): 2184–7. doi:10.1097/CCM.0b013e31817d7c71. PMID 18552679.
- ↑ Abgueguen, Pierre; Chennebault, Jean Marie; Pichard, Eric (2010). "Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome". The American Journal of Medicine. 123 (6): e3–4. doi:10.1016/j.amjmed.2009.09.034. PMID 20569743.
- ↑ Zipponi, Manuel; Eugster, Roland; Birrenbach, Tanja (2011). "High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome". BMJ Case Reports. 2011. doi:10.1136/bcr.12.2010.3599.
- ↑ Pecker, Mark; Adams, Michael; Graham, Walter (2011). "The Systemic Capillary Leak Syndrome: Comment". Annals of Internal Medicine. 155 (5): 335. doi:10.1059/0003-4819-155-5-201109060-00017. PMID 21893630.