scholarly journals Multiglandular Hormone Deficiency in a Patient with Systemic Capillary Leak Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Cornelia Then ◽  
Katrin Ritzel ◽  
Christa Seibold ◽  
Johannes F. E. Mann ◽  
Martin Reincke
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Primary Hypothyroidism ◽  
Interstitial Space ◽  
Capillary Leak Syndrome ◽  
Hormone Deficiency ◽  
Capillary Leak ◽  
Hormone Binding ◽  
Systemic Capillary Leak Syndrome ◽  
Peripheral Edema

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 to the point of hypovolemic shock. We report the case of a 64-year-old man with SCLS and multiple hormone abnormalities (primary hypothyroidism, hypoadrenalism, and hypogonadism), deficiency of hormone binding globulins, and hypogammaglobulinemia. The patient was successfully treated with intravenous immunoglobulins, theophylline, and terbutaline. Strikingly, with the dissolution of peripheral edema, hormone levels improved. To our knowledge, this is the first reported case of SCLS associated with polyglandular abnormalities.

Idiopathic systemic capillary leak syndrome (Clarkson’s disease) presenting with recurrent hypovolemic shock

2021 ◽  
Vol 20 (1) ◽  
pp. 74-77
Author(s):  
Kristen Davies ◽  
◽  
Kirsty Thomas ◽  
Lorna Barton ◽  
Chris Williams ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Compartment Syndromes ◽  
Monthly Basis ◽  
History Of ◽  
Clarkson’S Disease

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

Beneficial effect of intravenous immunoglobulins on systemic capillary leak syndrome in patients with monoclonal gammopathy

2009 ◽  
Vol 37 (2) ◽  
pp. 795
Author(s):  
Marc Lambert ◽  
David Launay ◽  
Eric Hachulla ◽  
Sandrine Morell-Dubois ◽  
Viviane Queyrel ◽  
...  
Keyword(s):  
Beneficial Effect ◽  
Monoclonal Gammopathy ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome

2017 ◽  
Vol 130 (10) ◽  
pp. 1219.e19-1219.e27 ◽  
Author(s):  
Marc Pineton de Chambrun ◽  
Marie Gousseff ◽  
Wladimir Mauhin ◽  
Jean-Christophe Lega ◽  
Marc Lambert ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Idiopatic Systemic Capillary Leak Syndrome Treated Successfully with High-Dose Intravenous Immunoglobulins

2015 ◽  
Vol 13 (2) ◽  
pp. 87-89
Author(s):  
Ferhat Ekinci ◽  
Utku Erdem Soyaltın ◽  
Harun Akar ◽  
Mehmet Can Ugur ◽  
Ercan Ersoy ◽  
...  
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Clinical Management ◽  
Relevant Literature ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

AbstractIdiopathic systemic capillary leak syndrome (ISCLS) is characterized by a triad of hypotension, hemoconcentration and hypoalbuminemia due to a shift of intravascular fluid and albumin to the extravascular area. We describe a hypovolemic patient with hemoconcentration and hypoalbuminemia who was successfully treated with high-dose intravenous immunoglobulins (IVIG). The purpose of this case report is to discuss the clinical management of idiopathic systemic capillary leak syndrome on the background of relevant literature review.

scholarly journals A Systemic Capillary Leak Syndrome (Clarkson Syndrome) in a Patient with Chronic Lymphocytic Leukemia: A Case Report in an Out-of-Hospital Setting

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Manon Durand Bechu ◽  
Antoine Rouget ◽  
Christian Recher ◽  
Elie Azoulay ◽  
Vincent Bounes
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
Poor Prognosis ◽  
Hypovolemic Shock ◽  
Hospital Setting ◽  
Capillary Leak Syndrome ◽  
Lymphocytic Leukemia ◽  
Fluid Replacement ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Initial Care

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. The disease can be idiopathic (Clarkson syndrome) or secondary to other diseases and treatments. We describe this syndrome in a prehospitalized, 63-year-old patient with chronic lymphocytic leukemia and an idiopathic form of SCLS manifesting as hypovolemic shock. Initial care is hospitalization in intensive care. In addition to etiological treatment if fluid replacement is necessary, treatment must be closely monitored for secondary overload complications. Catecholamine rather than arrhythmogenic support may be associated.

Idiopathic systemic capillary leak syndrome – An often missed diagnosis

2020 ◽  
pp. 004947552097929
Author(s):  
Remya Rajan ◽  
Nitin Kapoor ◽  
Hesarghatta S Asha ◽  
Thomas V Paul ◽  
Nihal Thomas
Keyword(s):  
Hypovolemic Shock ◽  
Capillary Leak Syndrome ◽  
Acute Episode ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Life Threatening ◽  
Missed Diagnosis

Idiopathic systemic capillary leak syndrome (ISCLS) is a potentially fatal disorder characterised by ‘attacks’ of varying intensity of hypovolemic shock in association with haemoconcentration and hypoalbuminaemia. It is a disease of exclusion, and the severity of attacks may mimic sepsis at presentation. We report a case of a lady with recurrent attacks of ISCLS with at least two life-threatening episodes, having been treated elsewhere as a case of steroid insufficiency. The diagnosis is often challenging, and treatment of an acute episode involves the judicious use of fluids and vasopressors, as required. Prophylaxis to prevent further attacks is of varied success.

Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report

2020 ◽  
Vol 49 (3) ◽  
pp. 372-378 ◽  
Author(s):  
Vittorio Di Maso ◽  
Martina Cozzi ◽  
Ugo Gerini ◽  
Elisa Bedina ◽  
Elisa Olivo ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Supportive Therapy ◽  
Intravenous Immunoglobulins ◽  
Abrupt Onset ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Plasma Filtration ◽  
Threatening Condition ◽  
Life Threatening ◽  
Exanthematous Pustulosis

Coupled plasma filtration adsorption (CPFA) is an extracorporeal supportive therapy based on nonspecific adsorption of pro- and anti-inflammatory mediators combined with continuous renal replacement therapy. The main field of CPFA application is septic shock, and there are limited data about its efficacy in the treatment of other acute conditions characterized by a dysregulation in immune homeostasis. Capillary leak syndrome (CLS) defines a life-threatening condition sustained by hypercytokinemia and characterized by abrupt onset of increased capillary permeability leading to severe generalized edema and hypovolemic shock refractory to fluid administration. Therapy for CLS is not specific and, at present time, it consists in the use of steroids or intravenous immunoglobulins. We present the case of a 34-year-old woman who developed CLS superimposed to acute generalized exanthematous pustulosis after initiating therapy with hydroxychloroquine for undifferentiated connective tissue disease. CLS did not respond to steroids and intravenous immunoglobulins, while it was successfully treated with CPFA. This observation supports the possible role of CPFA in restoring a proper immunologic homeostasis not only in sepsis but also in other devastating conditions sustained by hypercytokinemia.

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