Capillary leak syndrome: Difference between revisions
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A few [[prophylactic]] treatments have been tried with variable results, principally [[terbutaline]] and [[theophylline]]. | A few [[prophylactic]] treatments have been tried with variable results, principally [[terbutaline]] and [[theophylline]]. | ||
An episode of SCLS usually consists of two distinct phases: | An episode of SCLS usually consists of two distinct phases: | ||
===The capillary leak phase=== | ===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 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 (medicine)|syncope]] (fainting); limb, abdominal or generalized pain; facial or other [[edema]]; [[dyspnea]]; and [[hypotension]] that results in [[shock (circulatory)|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 (medicine)|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). | 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 (medicine)|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 syndrome|compartment syndromes]]. | ||
===The recruitment phase=== | ===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 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]].<ref>{{cite journal |pmid=1580299 |year=1992 |last1=Droder |first1=RM |last2=Kyle |first2=RA |last3=Greipp |first3=PR |title=Control of systemic capillary leak syndrome with aminophylline and terbutaline |volume=92 |issue=5 |pages=523–6 |journal=The American Journal of Medicine |doi=10.1016/0002-9343(92)90749-2}}</ref> The second, more recent approach pioneered in France involves monthly intravenous infusions of [[intravenous immunoglobulin]] (IVIG), and the growing case-report evidence is encouraging.<ref>{{cite journal |pages=2184–7 |doi=10.1097/CCM.0b013e31817d7c71 |title=High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome |year=2008 |last1=Lambert |first1=Marc |last2=Launay |first2=David |last3=Hachulla |first3=Eric |last4=Morell-Dubois |first4=Sandrine |last5=Soland |first5=Vincent |last6=Queyrel |first6=Viviane |last7=Fourrier |first7=François |last8=Hatron |first8=Pierre-Yves |journal=Critical Care Medicine |volume=36 |issue=7 |pmid=18552679}}</ref><ref>{{cite journal |pages=e3–4 |doi=10.1016/j.amjmed.2009.09.034 |title=Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome |year=2010 |last1=Abgueguen |first1=Pierre |last2=Chennebault |first2=Jean Marie |last3=Pichard |first3=Eric |journal=The American Journal of Medicine |volume=123 |issue=6 |pmid=20569743}}</ref><ref>{{cite journal|last1=Zipponi|first1=Manuel|last2=Eugster|first2=Roland|last3=Birrenbach|first3=Tanja|year=2011|title =High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome|journal=BMJ Case Reports|volume=2011|doi=10.1136/bcr.12.2010.3599|url=http://casereports.bmj.com/content/2011/bcr.12.2010.3599.abstract}}</ref><ref name="pecker">{{cite journal |first1=Mark |last1=Pecker |first2=Michael |last2=Adams |first3=Walter |last3=Graham |title=The Systemic Capillary Leak Syndrome: Comment |journal=Annals of Internal Medicine |pmid=21893630 |pages=335 |doi=10.1059/0003-4819-155-5-201109060-00017 |year=2011 |volume=155 |issue=5}}</ref> | 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]].<ref>{{cite journal |pmid=1580299 |year=1992 |last1=Droder |first1=RM |last2=Kyle |first2=RA |last3=Greipp |first3=PR |title=Control of systemic capillary leak syndrome with aminophylline and terbutaline |volume=92 |issue=5 |pages=523–6 |journal=The American Journal of Medicine |doi=10.1016/0002-9343(92)90749-2}}</ref> The second, more recent approach pioneered in France involves monthly intravenous infusions of [[intravenous immunoglobulin]] (IVIG), and the growing case-report evidence is encouraging.<ref>{{cite journal |pages=2184–7 |doi=10.1097/CCM.0b013e31817d7c71 |title=High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome |year=2008 |last1=Lambert |first1=Marc |last2=Launay |first2=David |last3=Hachulla |first3=Eric |last4=Morell-Dubois |first4=Sandrine |last5=Soland |first5=Vincent |last6=Queyrel |first6=Viviane |last7=Fourrier |first7=François |last8=Hatron |first8=Pierre-Yves |journal=Critical Care Medicine |volume=36 |issue=7 |pmid=18552679}}</ref><ref>{{cite journal |pages=e3–4 |doi=10.1016/j.amjmed.2009.09.034 |title=Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome |year=2010 |last1=Abgueguen |first1=Pierre |last2=Chennebault |first2=Jean Marie |last3=Pichard |first3=Eric |journal=The American Journal of Medicine |volume=123 |issue=6 |pmid=20569743}}</ref><ref>{{cite journal|last1=Zipponi|first1=Manuel|last2=Eugster|first2=Roland|last3=Birrenbach|first3=Tanja|year=2011|title =High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome|journal=BMJ Case Reports|volume=2011|doi=10.1136/bcr.12.2010.3599|url=http://casereports.bmj.com/content/2011/bcr.12.2010.3599.abstract}}</ref><ref name="pecker">{{cite journal |first1=Mark |last1=Pecker |first2=Michael |last2=Adams |first3=Walter |last3=Graham |title=The Systemic Capillary Leak Syndrome: Comment |journal=Annals of Internal Medicine |pmid=21893630 |pages=335 |doi=10.1059/0003-4819-155-5-201109060-00017 |year=2011 |volume=155 |issue=5}}</ref> | ||
Revision as of 18:54, 28 January 2013
| Capillary leak syndrome | |
| DiseasesDB | 34090 |
|---|---|
| MeSH | D019559 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and Keywords: Systemic Capillary Leak Syndrome, SCLS
Overview
Capillary Leak Syndrome is a rare medical condition where the number and size of the pores in the capillaries are increased which leads to a leakage of fluid from the blood to the interstitial fluid, resulting in dangerously low blood pressure (hypotension), edema and multiple organ failure due to limited perfusion.
Historical Perspective
The syndrome was first described by Clarkson in 1960.
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.
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.[1] The second, more recent approach pioneered in France involves monthly intravenous infusions of intravenous immunoglobulin (IVIG), and the growing case-report evidence is encouraging.[2][3][4][5]
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.[6]
Case Reports
Sources
- Dorlands Medical Dictionary 30th Ed.
- Cancer.gov
- Orphanet (2003)
References
- ↑ 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.
- ↑ 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.