Treatment is limited to withdrawal of growth factors and. Systemic capillary leak syndrome is a rare disorder characterized by repeated flares of massive leakage of plasma from blood vessels into neighboring body cavities and muscles. There is currently no cure for systemic capillary leak syndrome scls. Systemic capillary leak syndrome scls is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia this syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space even though scls has been. The systemic capillary leak syndrome scls is a rare disease of unknown etiology characterized by transient episodes of distributive shock. This may involve stabilizing the airway and breathing, taking certain medications, andor intravenous iv infusion of fluids, medications, or blood products. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. Systemic capillary leak syndrome genetic and rare diseases nih. In these circumstances, specific conditions such as shocklike syndrome, the need for vasopressors and capillary leak syndrome and laboratory criteria such as the levels of il6 and other. Capillary leak syndrome is characterized by the escape of blood plasma through capillary walls, from the blood circulatory system to surrounding tissues, muscle compartments, organs or body cavities.
Idiopathic systemic capillary leak syndrome iscls is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration. Capillary leak syndrome is a situation characterized by the escape of blood plasma through capillary walls, from the blood vessels to surrounding tissues, muscle compartments, organs or body cavities. Scls is characterized by hemoconcentation and hypoalbuminemia caused by reversible plasma extravasation. Vascular endothelial hyperpermeability induces the clinical. Idiopathic systemic capillary leak syndrome clarksons disease. Rapid improvement of lifethreatening capillary leak syndrome. Systemic inflammatory response syndrome sirs and ards in. It is associated with hemoconcentration, hypoalbuminemia, and generalized edema. Systemic capillary leak syndrome nord national organization. This can result in dangerously low blood pressure hypotensison, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. Systemic capillary leak syndrome care at mayo clinic.
The common pathogenies are sepsis, severe trauma, cardiopulmonary bypass and so on. Systemic capillary leak syndrome genetic and rare diseases. Systemic capillary leak syndrome scls, or clarksons disease, or primary capillary leak syndrome, is a rare, grave and episodic medical condition observed largely in otherwise healthy individuals mostly in middle age. Systemic capillary leak syndrome scls is a rare disorder characterized by acute and severe recurrent attacks associated with a rapid fall in blood pressure as a result of fluid leaks from smaller vessels called capillaries. Systemic capillary leak syndrome, the american journal of. Systemic capillary leak syndrome scls is a rare disorder characterized by increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space. Systemic capillary leak syndrome scls is a rare disease characterized by episodes of collapse due to rapid transfer of considerable volumes of plasma from the intravascular to the extravascular. Compartment syndrome as a result of systemic capillary. Systemic inflammatory response syndrome sirs and ards. Capillary leak syndrome is a rare and potentially lifethreatening condition caused by a shift of intravascular fluid and proteins to the interstitial space. Systemic capillary leak syndrome india pdf ppt case. Systemic capillary leak syndrome ireland pdf ppt case.
Systemic capillary leak syndrome is a rare disorder characterized by massive leakage of plasma and other blood components from blood vessels into neighboring body cavities and muscles. We report the case of a patient with idiopathic systemic capillary leak syndrome who developed an unexpected. High dose intravenous immunoglobulin therapy of the systemic. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. Pdf introduction the systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of hypotension due to rapid. This results in a sharp drop in blood pressure that, if not treated, can lead to organ failure and death. Capillary leak syndrome cls in patients with severe course of disease is more comand more mon, and the clinical manifestations of cls include systemic edema, hypoproteinemia, effective circulating blood volume reduction and hemoconcentration. Dec 07, 2010 the systemic capillary leak syndrome scls can be a dramatic, perplexing, and terrifying disease to treat, particularly in its initial presentation.
The aim of the study was to analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to identify contributing factors leading. Jul 10, 2009 the systemic capillary leak syndrome scls, clarkson syndrome is an exceedingly rare disorder of unknown cause characterized by chronic edema or acute transient, severe episodes of hypotension, hypovolemia, and oliguria. Systemic capillary leak syndrome scls is a rare but potentially fatal disorder characterized by a loss of fluid and proteins into the interstitial space leading to intravascular hypovolemia up. The cause is the sudden onset of capillary hyperpermeability with extravasations of plasma from the intravascular to the extravascular compartment. Systemic capillary leak syndrome scls, also named vascular leak syndrome vls or clarkson disease syndrome, was first reported by clarkson et al. Gemcitabineinduced systemic capillary leak syndrome annals.
Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome scls or clarksons dis. From the first publication in 1960, only 34 cases have been reported. High dose intravenous immunoglobulin therapy of the. Systemic capillary leak syndrome scls is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension. This results in diffuse swelling, weight gain, and renal shutdown. Mechanistic classification of the systemic capillary leak. Nov 06, 2018 systemic capillary leak syndrome scls is a rare disease characterized by shock caused by capillary hyperpermeability.
Systemic capillary leak syndrome is a rare disorder characterized by selfreversing episodes during which the endothelial cells which line the capillaries are thought to separate for a few days, allowing for massive leakage of plasma and other blood components from the blood vessels into neighbouring body cavities and muscles. The disease can be idiopathic clarkson syndrome or secondary to other diseases and treatments. This can result in dangerously low blood pressure hypotension, hypoalbuminemia, and a decrease in plasma volume hemoconcentration. In such cases, immediate withdrawal of retinoic acid is necessary and. Jul 31, 2017 there is currently no cure for systemic capillary leak syndrome scls. Systemic capillary leak syndrome associated with hypovolemic. We describe this syndrome in a prehospitalized, 63yearold patient with. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Scls is difficult to diagnose prospectively because the clinical criteriahypotension, hemoconcentration, low serum albuminare nonspecific and not present in all cases.
Idiopathic systemic capillary leak syndrome in childhood. Systemic leakage syndrome causes complex problems involving many organs and organ systems. Systemic capillary leak syndrome doctors and departments. Systemic capillary leak syndrome active research protocols. In various human diseases, an increase in capillary permeability to proteins leads to the loss of proteinrich fluid from the intravascular to the interstitial space. Several treatment methods are used for systemic capillary leak syndrome.
Capillary leak syndrome radiology reference article. A direct correlation between il11 and cls has never been reported previously, particularly in patients with hepatic carcinoma. A systemic capillary leak syndrome clarkson syndrome in a. Systemic capillary leak syndrome clarkson syndrome in cancer patients. Systemic capillary leak syndrome scls is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia this syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space even though scls has been suggested to. We have treated a patient with recurrent scls and have consulted by telephone on another patient. Massive capillary leak ards ascites pleural effusions 4lfts thrombocytopenia.
The systemic capillary leak syndrome scls, clarkson syndrome is an exceedingly rare disorder of unknown cause characterized by chronic edema or acute transient, severe episodes of hypotension, hypovolemia, and oliguria. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. Systemic capillary leak syndrome, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset 17 mar 2017 diseases other than sepsis that can result in capillary leak syndrome include the. Interleukin11 il11 is a promising therapeutic agent for thrombocytopenia. Capillary leak syndrome after interferon treatment. This syndrome is characterised by generalised edema, hypotension, hemoconcentration, and hypoproteinemia. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004. Mar 17, 2016 aka clarksons disease leaky capillary disease. Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in.
Systemic capillary leak syndrome and monoclonal igg gammopat. There is no curative treatment, thus management is symptomatic and prophylactic. The heparan sulfate proteoglycan of the glomerular basement membrane is thought to be a key element to retain erythrocytes and plasma protein in circulation. Immunoglobulins for treatment of systemic capillary leak syndrome.
In 1960, dr bayard clarkson described a patient who experienced sporadic bouts of hypovolemia, hypotension, and edema. The increase in capillary permeability facilitates the movement of proteinrich fluid from the intravascular to. The causes of idiopathic systemic capillary leak syndrome iscls is. Systemic capillary leak syndrome symptoms and causes mayo. Apr 01, 2010 capillary leak syndrome cls is a severe complication of allogeneic stem cell transplantation sct characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia. Systemic capillary leak syndrome scls, also named vascular leak syndrome vls or clarkson diseasesyndrome, was first reported by clarkson et al. Systemic capillary leak syndrome symptoms and causes.
To describe a single case of systemic capillary leak syndrome scls with a rare complication of compartment syndrome. Systemic capillary leak syndrome pdf gelmek learn how. If treatment is not done massive fluid shifts result in loss of blood pressure which can lead to. Capillary leak syndrome ankita mehta, md, and mansi shah, md introduction capillary leak syndrome cls is a rare clinical disease that causes edema, hypoproteinemia, episodic hypotension, dyspnea, hyponatremia, and weight gain that can be life threatening 1. Interleukin11induced capillary leak syndrome in primary. Systemic capillary leak syndrome scls is a rare disease with poor prognosis, characterized by the occurrence of mucocutaneous and visceral edema with hypotension, hemoconcentration, and unexpected hypoalbuminemia. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a sepsislike syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions. Systemic capillary leak syndrome is a rare disease with a high mortality rate.
The systemic capillary leak syndrome scls can be a dramatic, perplexing, and terrifying disease to treat, particularly in its initial presentation. Systemic capillary leak syndrome scls is a rare disease characterized by shock caused by capillary hyperpermeability. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for systemic capillary leak. It is associated with transient, recurrent episodes of capillary hyperpermeability that lead to the seepage of fluids and proteins from the intravascular to the extravascular compartments.
Treatment is directed at prevention of attacks using agents aimed at decreasing capillary leakage and aimed at. Treatment for scls during an episode is mainly supportive, aiming to stabilize symptoms and prevent severe complications. Studies in the pathogenesis of systemic capillary leak. During attacks of iscls, profound derangements of the vascular endothelium develop, resulting in leakage of plasma and proteins into the interstitial compartment 1,2.
Severe edema results from leakage of fluid and macromolecules 200900 kda into tissues. Systemic capillary leak syndrome is a rare and lifethreatening disorder characterized by hypotension that can lead to shock, weight gain, hypoalbuminemia, and elevated hematocrit secondary to unexplained episodic capillary fluid extravasation into the interstitial space. An episode may include sudden drop in blood pressure. A systemic capillary leak syndrome clarkson syndrome in. Systemic capillary leak syndrome definition of systemic. Immunoglobulins for treatment of systemic capillary leak. Capillary leak syndrome cls is a severe complication of allogeneic stem cell transplantation sct characterized by weight gain, generalized edema, hypotension, and hypoalbuminemia. Capillary leak syndrome an overview sciencedirect topics. Systemic capillary leak syndrome pdf gelmek learn how to. The systemic capillary leak syndrome annals of internal. Capillary leak syndrome cls is a rare condition characterized by recurrent episodes of generalized edema and severe hypotension associated with hypoproteinemia. Rapid improvement of lifethreatening capillary leak. Jun 16, 2014 systemic capillary leak syndrome is a rare and life threatening disease characterized by periodic episodes of hypovolemic shock due to leakage of plasma from the intravascular to the extravascular space. The systemic capillary leak syndrome is a highly rare disorder of unknown etiology.
Systemic capillary leak syndrome scls proposed approach. May 03, 2012 in 1960, dr bayard clarkson described a patient who experienced sporadic bouts of hypovolemia, hypotension, and edema. Idiopathic systemic capillary leak syndrome uptodate. Summary systemic capillary leak syndrome scls is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia this syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space even though scls has been. Previous treatment of this potentially devastating. From the laboratory of clinical investigation, national institute of allergy and infectious diseases and the metabolism and immunology branches, national cancer institute, national institutes of health. These conditions and factors are sources of secondary capillary leak syndrome. Jul 31, 2017 systemic capillary leak syndrome scls is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues. Clinical manifestations of capillary leak syndrome. Capillary leak syndrome article about capillary leak. Systemic capillary leak syndrome scls is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues.
A patient with pustular psoriasis developed capillary leak syndrome after the start of acitretin therapy 35 a. Also called clarksons disease, this condition can be mistaken. Systemic inflammatory response syndrome sirs and ards in the picu background and definitions pathophysiology novel approaches andrew t. Gemcitabineinduced systemic capillary leak syndrome. Our patient is a 57yearold male, referred to our hospital due to polycythemia hemoglobin hgb of 220 gl, hypotension, acute renal failure, and bilateral calf pain. Idiopathic systemic capillary leak syndrome scls, also known as clarksons disease, and spontaneous periodic edema is a very rare and lifethreatening disorder first described in 1960 by clarkson et al. Systemic capillary leak syndrome scls is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. Compartment syndrome as a result of systemic capillary leak. Systemic capillary leak syndrome clarkson syndrome in. Gemcitabineinduced systemic capillary leak syndrome pdf. Systemic capillary leak syndrome scls is very rare and lethal disease and only 150 cases have been reported after the first publication of its report in 1960 by clarkson. An analogous and more common case is renal proteinuria. Studies in the pathogenesis of systemic capillary leak syndrome. We describe this syndrome in a prehospitalized, 63yearold patient with chronic.