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'''For patient information, click [[Capillary leak syndrome (patient information)|here]]'''
'''For patient information, click [[Capillary leak syndrome (patient information)|here]]'''


{{CMG}}
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [https://www.wikidoc.org/index.php/User:Hassan_M M. Hassan, M.B.B.S]
{{Capillary leak syndrome}}
{{Capillary leak syndrome}}


'''''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]]==
 
==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==
The episode usually consists of two phases


1. The capillary leak phase (1-4 days)
==[[Capillary leak syndrome historical perspective|Historical Perspective]]==
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 pathophysiology|Pathophysiology]]==
==[[Capillary leak syndrome causes|Causes]]==


2. Recruitment of the interstitial fluid
==[[Capillary leak syndrome differential diagnosis|Differentiating Capillary leak syndrome from other Diseases]]==
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]].
==[[Capillary leak syndrome epidemiology and demographics|Epidemiology and Demographics]]==


==Prognosis==
==[[Capillary leak syndrome risk factors|Risk Factors]]==
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%.<ref name="druey"/><ref name="kapoor"/> 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.<ref name="gousseff" /> 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.
==[[Capillary leak syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==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===
==Treatment==
* Hemoconcentration
* [[Hypoalbuminemia]] without [[albuminuria]]


==External Links==
[[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]]
*[http://ndt.oxfordjournals.org/cgi/content/full/17/3/492 Case report in Oxford Journals]
* [http://www.cancer.gov/Templates/db_alpha.aspx?CdrID=426421 Cancer.gov]


==References==
==Case Studies==
{{Reflist|2}}
[[Capillary leak syndrome case study one|Case #1]]


[[Category:Syndromes]]
[[Category:Syndromes]]
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Latest revision as of 18:09, 16 November 2022


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Synonyms and Keywords: Systemic capillary leak syndrome; SCLS; Clarkson's disease.

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Capillary leak syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

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Case #1


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