scholarly journals Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase

2017 ◽  
Vol 07 (02) ◽  
pp. 094-096 ◽  
Author(s):  
Ashley Bjorklund ◽  
Gwenyth Fisher ◽  
Anna Sofi Asmundsson
Keyword(s):  
Intravenous Immunoglobulin ◽  
Acute Phase ◽  
Clinical Course ◽  
Pediatric Intensive Care ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Acute Therapy ◽  
Vasopressor Support ◽  
Distributive Shock

AbstractSystemic capillary leak syndrome (SCLS) is a potentially life-threatening disorder characterized by distributive shock, hypoalbuminemia, and hemoconcentration. It is exceedingly rare in children with less than 20 cases reported to date. The underlying cause for this syndrome remains largely unknown and acute treatment has remained mainly supportive. Prophylaxis with intravenous immunoglobulin (IVIG) has been shown to successfully prevent further episodes in both adults and children. We present a case of a 2-year-old previously healthy male admitted to the pediatric intensive care unit with a clinical course consistent with SCLS. His shock was refractory to aggressive fluid and vasopressor support. Reversal of SCLS with IVIG given in the acute phase had been described in three adult subjects, and for this reason, the decision was made to administer IVIG. Within an hour of administration, hemodynamics stabilized and vasopressor support could be weaned. He has had no further episodes on prophylactic infusions of IVIG. Although the exact mechanism of IVIG in SCLS is unknown, it has proven to be an effective and safe prophylactic therapy, and in our patient, it drastically reversed the acute capillary leak. We suggest that IVIG should be considered as acute therapy in pediatric patients with refractory shock and a clinical course suggestive of SCLS.

scholarly journals Autonomic biomarkers of shock in idiopathic systemic capillary leak syndrome

PLoS ONE ◽  
2021 ◽  
Vol 16 (6) ◽  
pp. e0251775
Author(s):  
Maddalena Alessandra Wu ◽  
Emanuele Catena ◽  
Antonio Castelli ◽  
Roberto Rech ◽  
Beatrice Borghi ◽  
...  
Keyword(s):  
Heart Rate ◽  
Acute Phase ◽  
Systolic Arterial Pressure ◽  
Recovery Phase ◽  
Capillary Leak Syndrome ◽  
Haemodynamic Instability ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Baroreflex Control ◽  
Distributive Shock

Objective The term Idiopathic Systemic Capillary Leak Syndrome (ISCLS) refers to an uncommon condition of severe distributive shock, resulting from an abrupt shift of fluids and proteins from the intravascular to the interstitial compartment. We hypothesise that the autonomic nervous system (ANS) fails in regulating the response to hypovolemia in acute ISCLS and that ANS variables characterise the progression to the recovery. Design Prospective cohort study of patients admitted to ICU for severe ISCLS flares. Setting Single, referral center in Italy for ISCLS. Patients Analysis of cardiovascular signals recorded during seven severe ISCLS attacks and one prodromal period in five patients. Interventions ANS was studied non-invasively by means of heart rate variability (HRV) and blood pressure variability analysis, as an estimation of vagal and sympathetic modulation directed to the heart and vessels. Heart rate and systolic arterial pressure (SAP) variability were also used to assess baroreflex sensitivity. ANS variables were measured during the subsequent phases which characterise ISCLS flares, namely the acute phase, the post-acute phase, and the recovery phase. Measurements and main results HRV was severely depressed during the acute phase accounting for the loss of ANS modulation during massive capillary extravasation. This phase was characterised by shock and impaired baroreflex control, which allowed SAP to oscillate driven by respiratory activity. Impending shock and transition from shock to a post-acute phase were marked by change of baroreflex spectral variables. The baroreflex control was fully restored during recovery. Conclusions ANS modulation and baroreflex control are severely impaired during the acute haemodynamic instability which characterises ISCLS crises and their progressive restoration may be a clue of improvement. ANS indices during ISCLS flares might serve as useful biomarkers, able to timely announce the transition from one phase to the subsequent one, thus helping to adapt therapy accordingly.

scholarly journals Hyperviscosity-related splenic infarction, gastrointestinal ischaemia–reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson’s syndrome)

2020 ◽  
Vol 13 (1) ◽  
pp. e232500
Author(s):  
Jelle Alexander van Erven ◽  
Jolanda Schrama ◽  
Daan Albert Robertus Castelijn
Keyword(s):  
Splenic Infarction ◽  
Capillary Leak Syndrome ◽  
Polycythaemia Vera ◽  
Capillary Leak ◽  
Myeloproliferative Disease ◽  
Systemic Capillary Leak Syndrome ◽  
Ischaemia Reperfusion Injury ◽  
Correct Treatment ◽  
Ischaemia Reperfusion ◽  
Distributive Shock

Clarkson’s syndrome, also known as idiopathic systemic capillary leak syndrome, is characterised by vascular hyperpermeability resulting in intravascular hypovolaemia and shock. A clinician should consider the diagnosis if other causes of shock, for example, sepsis and anaphylaxis, are ruled out and concomitant hyperviscosity is not caused by a myeloproliferative disease. Here, we describe a patient presenting with severe plasma leakage and assumable blood hyperviscosity leading to splenic infarction, gastrointestinal ischaemia–reperfusion syndrome and transient dysarthria. Our patient was first suspected of polycythaemia vera and phlebotomies were performed. Awareness of this syndrome and subsequent correct treatment is essential to prevent complications and to reduce mortality. As in our patient, most patients with Clarkson’s syndrome have a monoclonal gammopathy, light-chain-type kappa. Prophylactic treatment with intravenous immunoglobulin (IVIg) is advised to prevent recurrence of capillary leak. Our patient did not suffer from another symptomatic episode after starting IVIg.

scholarly journals Distributive shock due to systemic capillary leak syndrome treated with high-dose immunosuppression

2013 ◽  
Vol 2013 (apr09 1) ◽  
pp. bcr2013009048-bcr2013009048 ◽  
Author(s):  
J. R. Sheehan ◽  
L. Keating ◽  
A. Chan ◽  
A. Walden
Keyword(s):  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Distributive Shock

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

Immunotherapy ◽  
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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