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

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.

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

BANTAO Journal ◽

10.1515/bj-2015-0019 ◽

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.

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High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome

BMJ Case Reports ◽

10.1136/bcr.12.2010.3599 ◽

2011 ◽

Vol 2011 (apr27 1) ◽

pp. bcr1220103599-bcr1220103599 ◽

Cited By ~ 10

Author(s):

M. Zipponi ◽

R. Eugster ◽

T. Birrenbach

Keyword(s):

Therapeutic Approach ◽

Intravenous Immunoglobulins ◽

Capillary Leak Syndrome ◽

High Dose ◽

Capillary Leak ◽

Systemic Capillary Leak Syndrome ◽

Promising Therapeutic Approach

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High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome

Critical Care Medicine ◽

10.1097/ccm.0b013e31817d7c71 ◽

2008 ◽

Vol 36 (7) ◽

pp. 2184-2187 ◽

Cited By ~ 64

Author(s):

Marc Lambert ◽

David Launay ◽

Eric Hachulla ◽

Sandrine Morell-Dubois ◽

Vincent Soland ◽

...

Keyword(s):

Intravenous Immunoglobulins ◽

Capillary Leak Syndrome ◽

High Dose ◽

Capillary Leak ◽

Systemic Capillary Leak Syndrome

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Multiglandular Hormone Deficiency in a Patient with Systemic Capillary Leak Syndrome

Case Reports in Medicine ◽

10.1155/2015/958283 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6 ◽

Cited By ~ 2

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.

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Nivolumab induced systemic capillary leak syndrome as an ultra rare life-threatening phenomenon of late toxicity and intravenous immunoglobulin efficacy

Immunotherapy ◽

10.2217/imt-2020-0335 ◽

2021 ◽

Author(s):

Ilya Polishchuk ◽

Alexander Yakobson ◽

Melanie Zemel ◽

Adam A Sharb ◽

Walid Shalata ◽

...

Keyword(s):

Intravenous Immunoglobulin ◽

Hypovolemic Shock ◽

Late Toxicity ◽

Immunoglobulin Therapy ◽

Capillary Leak Syndrome ◽

High Dose ◽

Capillary Leak ◽

Systemic Capillary Leak Syndrome ◽

Intravenous Immunoglobulin Therapy ◽

Life Threatening

Systemic capillary leak syndrome (SCLS) is a life-threatening disease. It is characterized by severe capillary hyperpermeability to proteins resulting in hemoconcentration, hypoalbuminemia and hypovolemic shock. Its treatment remains supportive, and the prognosis is generally poor. We report on a 51-year old male with melanoma treated with nivolumab for 1 year. 1 month following the completion of the treatment, the patient presented with signs of hypovolemic shock, anasarca, hemoconcentration and hypoalbuminemia. After excluding other diseases, a diagnosis of nivolumab-induced systemic capillary leak syndrome was made. A high dose of intravenous steroid therapy was promptly initiated without any significant clinical improvement. Intravenous immunoglobulin therapy was then administered with normalization of blood pressure, hemoconcentration and complete resolution of anasarca. Intravenous immunoglobulin should be considered a first-line treatment option for this rare phenomenon.

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Capillary leak syndrome (CLS) from rituximab therapy of lymphoma

Journal of Cancer Science and Therapeutics ◽

10.36879/jcst.20.000118 ◽

2020 ◽

pp. 1-3

Keyword(s):

Chemotherapy Regimen ◽

Capillary Leak Syndrome ◽

Plasma Extravasation ◽

Capillary Leak ◽

Radiological Findings ◽

Systemic Capillary Leak Syndrome ◽

Drug Induced ◽

Multi Agent ◽

Clarkson’S Disease

Capillary Leak Syndome (CLS) is characterized by plasma extravasation into the interstitium with resultant hypotension, anasarca, hemoconcentration, and hypoalbuminemia in the absence of albuminuria. Initially reported in Clarkson’s disease (systemic capillary leak syndrome, SCLS), CLS has been observed in multiple disease settings, the most common being sepsis. In Oncology, CLS has been reported more often as a complication from therapy, and less often from malignancy. In this case study, we documented clinical manifestation, laboratory features and radiological findings of CLS from rituximab therapy when employed in combination with a multi-agent chemotherapy regimen (EPOCH-R). Differentiating drug-induced CLS from sepsis, which presents with the same clinical features, is important in avoiding further exposure to rituximab, which could be fatal to the patient.

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