|
|
| (27 intermediate revisions by 3 users not shown) |
| Line 1: |
Line 1: |
| {{Infobox_Disease
| | __NOTOC__ |
| | Name = {{PAGENAME}}
| | |
| | Image =
| | '''For patient information, click [[Capillary leak syndrome (patient information)|here]]''' |
| | Caption =
| | |
| | DiseasesDB = 34090
| | {{CMG}}; '''Associate Editor(s)-In-Chief:''' [https://www.wikidoc.org/index.php/User:Hassan_M M. Hassan, M.B.B.S] |
| | ICD10 =
| | {{Capillary leak syndrome}} |
| | ICD9 =
| |
| | ICDO =
| |
| | OMIM =
| |
| | MedlinePlus =
| |
| | eMedicineSubj =
| |
| | eMedicineTopic =
| |
| | MeshID = D019559
| |
| }}
| |
| {{SI}} | |
| {{CMG}} | |
|
| |
|
| '''''Synonyms and Keywords:''''' Systemic capillary leak syndrome; SCLS; Clarkson's disease. | | '''''Synonyms and Keywords:''''' Systemic capillary leak syndrome; SCLS; Clarkson's disease. |
|
| |
|
| ==Overview== | | ==[[Capillary leak syndrome overview|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 (biology)|interstitial space]], resulting in a dangerous [[hypotension]] (low blood pressure), [[hemoconcentration#Elevated|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,<ref name="clarkson">{{cite journal |doi=10.1016/0002-9343(60)90018-8 |title=Cyclical edema and shock due to increased capillary permeability |year=1960 |last1=Clarkson |first1=Bayard |last2=Thompson |first2=David |last3=Horwith |first3=Melvin |last4=Luckey |first4=E.Hugh |journal=The American Journal of Medicine |volume=29 |issue=2 |pmid=13693909 |pages=193–216}}</ref> 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.<ref name="druey">{{cite journal |first1=Kirk M. |last1=Druey |first2=Philip R. |last2=Greipp |title=Narrative Review: Clarkson Disease-Systemic Capillary Leak Syndrome |journal=Annals of Internal Medicine |pmid=20643990 |pages=90–8 |doi=10.1059/0003-4819-153-2-201007200-00005 |pmc=3017349 |year=2010 |volume=153 |issue=2}}</ref><ref name="kapoor">{{cite journal |pages=905–12 |doi=10.4065/mcp.2010.0159 |pmc=2947962 |title=Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience |year=2010 |last1=Kapoor |first1=Prashant |last2=Greipp |first2=Patricia T. |last3=Schaefer |first3=Eric W. |last4=Mandrekar |first4=Sumithra J. |last5=Kamal |first5=Arif H. |last6=Gonzalez-Paz |first6=Natalia C. |last7=Kumar |first7=Shaji |last8=Greipp |first8=Philip R. |journal=Mayo Clinic Proceedings |volume=85 |issue=10 |pmid=20634497}}</ref>
| |
|
| |
|
| ==Natural History== | | ==[[Capillary leak syndrome historical perspective|Historical Perspective]]== |
| The episode usually consists of two phases
| | ==[[Capillary leak syndrome pathophysiology|Pathophysiology]]== |
| | ==[[Capillary leak syndrome causes|Causes]]== |
|
| |
|
| 1. The capillary leak phase (1-4 days)
| | ==[[Capillary leak syndrome differential diagnosis|Differentiating Capillary leak syndrome from other Diseases]]== |
| 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]].
| | ==[[Capillary leak syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
|
| |
|
| 2. Recruitment of the interstitial fluid
| | ==[[Capillary leak syndrome risk factors|Risk Factors]]== |
| 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== | | ==[[Capillary leak syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.
| |
|
| |
|
| ==Diagnosis== | | ==Diagnosis== |
| ===Symptoms===
| |
| [[Generalized swelling]] may be present.
| |
|
| |
| ===Physical Examination===
| |
| ====Vitals====
| |
| * [[Low blood pressure]] ([[hypotension]])
| |
|
| |
|
| ====Skin====
| | [[Capillary leak syndrome history and symptoms|History and Symptoms]] | [[Capillary leak syndrome physical examination|Physical Examination]] | [[Capillary leak syndrome laboratory findings|Laboratory Findings]] | [[Capillary leak syndrome other diagnostic studies|Other Diagnostic Studies]] |
| Generalized [[edema]] or [[anasarca]]
| |
| | |
| ===Laboratory Studies===
| |
| * Hemoconcentration
| |
| * [[Hypoalbuminemia]] without [[albuminuria]]
| |
|
| |
|
| ==Treatment== | | ==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 (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). 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 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>
| |
|
| |
| 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.<ref name="gousseff">{{cite journal |first1=Marie |last1=Gousseff |first2=Laurent |last2=Arnaud |first3=Marc |last3=Lambert |first4=Arnaud |last4=Hot |first5=Mohamed |last5=Hamidou |first6=Pierre |last6=Duhaut |first7=Thomas |last7=Papo |first8=Martin |last8=Soubrier |first9=Marc |last9=Ruivard |title=The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry |journal=Annals of Internal Medicine |pmid=21464348 |pages=464–71 |doi=10.1059/0003-4819-154-7-201104050-00004 |year=2011 |volume=154 |issue=7}}</ref>
| |
|
| |
| ==Case Reports==
| |
| *[http://ndt.oxfordjournals.org/cgi/content/full/17/3/492 Case report in Oxford Journals]
| |
|
| |
|
| ==Sources==
| | [[Capillary leak syndrome medical therapy|Medical Therapy]] | [[Capillary leak syndrome prevention|Prevention]] | [[Capillary leak syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Capillary leak syndrome future or investigational therapies|Future or Investigational Therapies]] |
| * Dorlands Medical Dictionary 30th Ed.
| |
| * [http://www.cancer.gov/Templates/db_alpha.aspx?CdrID=426421 Cancer.gov]
| |
| * [http://www.orpha.net/static/GB/capillary_leak_syndrome.html Orphanet (2003)]
| |
|
| |
|
| ==References== | | ==Case Studies== |
| {{Reflist|2}}
| | [[Capillary leak syndrome case study one|Case #1]] |
|
| |
|
| [[Category:Syndromes]] | | [[Category:Syndromes]] |
| [[Category:Intensive care medicine]] | | [[Category:Intensive care medicine]] |
| [[Category:Emergency medicine]] | | [[Category:Emergency medicine]] |
| | [[Category:Disease]] |
|
| |
|
| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
| {{WS}}
| |