Capillary Leak Syndrome

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Pierre-yves Hatron - One of the best experts on this subject based on the ideXlab platform.

Kirk M Druey - One of the best experts on this subject based on the ideXlab platform.

  • neutrophil activation in systemic Capillary Leak Syndrome clarkson disease
    Journal of Cellular and Molecular Medicine, 2019
    Co-Authors: Douglas B Kuhns, Xuesong Gu, Towia A Libermann, John I Gallin, Samir M Parikh, Kirk M Druey
    Abstract:

    : Systemic Capillary Leak Syndrome (SCLS; Clarkson disease) is a rare orphan disorder characterized by transient yet recurrent episodes of hypotension and peripheral oedema due to diffuse vascular Leakage of fluids and proteins into soft tissues. Humoral mediators, cellular responses and genetic features accounting for the clinical phenotype of SCLS are virtually unknown. Here, we searched for factors altered in acute SCLS plasma relative to matched convalescent samples using multiplexed aptamer-based proteomic screening. Relative amounts of 612 proteins were changed greater than twofold and 81 proteins were changed at least threefold. Among the most enriched proteins in acute SCLS plasma were neutrophil granule components including bactericidal permeability inducing protein, myeloperoxidase and matrix metalloproteinase 8. Neutrophils isolated from blood of subjects with SCLS or healthy controls responded similarly to routine pro-inflammatory mediators. However, acute SCLS sera activated neutrophils relative to remission sera. Activated neutrophil supernatants increased permeability of endothelial cells from both controls and SCLS subjects equivalently. Our results suggest systemic neutrophil degranulation during SCLS acute flares, which may contribute to the clinical manifestations of acute vascular Leak.

  • severe Capillary Leak Syndrome with cardiac arrest triggered by influenza virus infection
    Case Reports, 2018
    Co-Authors: Lotte Ebdrup, Kirk M Druey, Trine H Mogensen
    Abstract:

    Systemic Capillary Leak Syndrome (SCLS), also known as Clarkson Syndrome, is a rare disease with potential fatal outcome. The clinical picture involves Leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension. The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock. We present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment Syndrome. The severe episode of Capillary Leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode. Here we describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.

  • idiopathic systemic Capillary Leak Syndrome clarkson disease
    The Journal of Allergy and Clinical Immunology, 2017
    Co-Authors: Kirk M Druey, Samir M Parikh
    Abstract:

    In 1960, Dr Bayard Clarkson described a woman experiencing sporadic recurrent episodes of shock and anasarca. Plasma from an acute attack induced a shock-like Syndrome when injected into rats. The enigmatic systemic Capillary Leak Syndrome (SCLS) named for Dr Clarkson is characterized by transient and severe but reversible hemoconcentration and hypoalbuminemia caused by Leakage of fluids and macromolecules into tissues. Although less than 500 cases of SCLS have been reported in the literature since 1960, the condition is probably underdiagnosed because of a lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis because its presentation can resemble more common plasma Leakage Syndromes, including angioedema or systemic anaphylaxis. Although the precise molecular cause of SCLS remains unknown, substantial advances over the last 5 years have increased our understanding of SCLS pathogenesis.

  • the role of monoclonal paraproteins in systemic Capillary Leak Syndrome scls
    Journal of Immunology, 2016
    Co-Authors: Reem M Hakeem, Kirk M Druey, Xie Zhihui
    Abstract:

    The Systemic Capillary Leak Syndrome (SCLS) is a rare, orphan disease characterized by bouts of acute, reversible plasma extravasation into tissues, which leads to hemoconcentration, hypoalbuminemia, hypotension, and vascular collapse. Although the cause of SCLS is unknown, 80–95 % of SCLS patients have a monoclonal gammopathy of unknown significance (MGUS), in which clonal plasma cells secretes large amounts of monoclonal immunoglobulins or “paraproteins”. MGUS is a premalignant precursor to multiple myeloma (MM) and is notably detected in most SCLS patient sera. Another marked distinction is that acute, but not basal SCLS sera contain elevated concentrations of inflammatory mediators known to promote endothelial permeability, notably Vascular Endothelial Growth Factor (VEGF) and Angiopoietin 2 (Angpt2). To address these findings experimentally, we purified whole IgG from patient and control sera to examine whether these immunoglobulins have an impact on endothelial cell barrier function and performed functional assays to characterize the observed changes in morphology. Preliminary data suggest that patient IgG binds to endothelial cells and elicits significant F-actin stress fiber formation and VE-cadherin internalization. SCLS IgG seems to be critical in triggering endothelial dysfunction and dissecting the underlying signaling mechanisms involved will help us better understand disease pathogenesis and potentially pave the way for therapeutic target development.

  • Narrative Review: The Systemic Capillary Leak Syndrome
    2015
    Co-Authors: Kirk M Druey
    Abstract:

    The systemic Capillary Leak Syndrome (SCLS) is a rare disease of reversible plasma extravasation and vascular collapse accom-panied by hemoconcentration and hypoalbuminemia. Its cause is unknown, although it is believed to be a manifesta-tion of transient endothelial dysfunction due to endothelial contraction, apoptosis, injury, or a combination of these. Fewer than 150 cases of SCLS have been reported, but the condition is probably underrecognized because of its nonspe-cific symptoms and signs and high mortality rate. Patients experience shock and massive edema, often after a nonspe-cific prodrome of weakness, fatigue, and myalgias, and are at risk for ischemia-induced organ failure, rhabdomyolysis and muscle compartment Syndromes, and venous thromboembo-lism. Shock and edema reverse almost as quickly as they begin, at which time patients are at risk for death from flash pulmonary edema during rapid fluid remobilization. Diagnosis is made clinically and by exclusion of other diseases that cause similar symptoms and signs, most notably sepsis, ana-phylaxis, and angioedema. Acute episodes are treated with vasopressor therapy and judicious fluid replacement, possibly with colloid solutions for their osmotic effects, to prevent the sequelae of underperfusion. Between episodes, patients may be treated with theophylline and terbutaline, which clinical experience suggests may reduce the severity and frequency of acute episodes. Prognosis is uncertain, but patients who sur-vive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%. Much remains to be learned about SCLS, and clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension. Ann Intern Med. 2010;153:90-98. www.annals.or

Marc Lambert - One of the best experts on this subject based on the ideXlab platform.

  • intravenous immunoglobulins improve survival in monoclonal gammopathy associated systemic Capillary Leak Syndrome
    The American Journal of Medicine, 2017
    Co-Authors: Marc Pineton De Chambrun, Marc Lambert, Wladimir Mauhin, Marie Gousseff, J C Lega, Sophie Riviere, Antoine Dossier, Marc Ruivard, Francois Lhote, Gilles Blaison
    Abstract:

    Abstract Background Monoclonal gammopathy-associated systemic Capillary-Leak Syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of Capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic Capillary-Leak Syndrome. Methods We conducted a cohort analysis of all patients included in the European Clarkson disease (EureClark) registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (eg, the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months. Results Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%). Median (interquartile range) follow-up duration was 5.1 (2.5-9.7) years. Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Multivariate analysis found preventive treatment with IVIg (hazard ratio 0.27; 95% confidence interval, 0.10-0.70; P  = .007) and terbutaline (hazard ratio 0.35; 95% confidence interval, 0.13-0.96; P  = .041) to be independent predictors of mortality. Conclusions We describe the largest cohort to date of patients with well-defined monoclonal gammopathy-associated systemic Capillary-Leak Syndrome. Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy.

  • subcutaneous immunoglobulin therapy prevents systemic Capillary Leak Syndrome attack
    The American Journal of Medicine, 2016
    Co-Authors: Quentin Scanvion, Eric Hachulla, Pierre-yves Hatron, Guillaume Lefevre, Marc Lambert
    Abstract:

    We report the first case of systemic Capillary Leak Syndrome successfully treated with subcutaneous immunoglobulins.

  • the systemic Capillary Leak Syndrome a case series of 28 patients from a european registry
    Annals of Internal Medicine, 2011
    Co-Authors: M Gousseff, Marc Lambert, Laurent Arnaud, Arnaud Hot, Mohamed Hamidou, P Duhaut, T Papo, Martin Soubrier, M Ruivard, Giuseppe Malizia
    Abstract:

    The systemic Capillary Leak Syndrome (SCLS) is a rare condition characterized by unexplained episodic attacks of systemic Capillary hyperpermeability that can lead to fatal pulmonary edema. Optimal...

  • high dose intravenous immunoglobulins dramatically reverse systemic Capillary Leak Syndrome
    Critical Care Medicine, 2008
    Co-Authors: Marc Lambert, Eric Hachulla, S Morelldubois, Vincent Soland, Francois Fourrier, V. Queyrel, David Launay, Pierre-yves Hatron
    Abstract:

    Objective:The objective of this study was to report the dramatic improvement of patients with systemic Capillary Leak Syndrome obtained with high-dose intravenous immunoglobulins.Design:Systemic Capillary Leak Syndrome is a rare and life-threatening disorder characterized by hypotension that can lea

  • high dose intravenous immunoglobulins dramatically reverse systemic Capillary Leak Syndrome
    Critical Care Medicine, 2008
    Co-Authors: Marc Lambert, Eric Hachulla, S Morelldubois, Vincent Soland, Francois Fourrier, V. Queyrel, David Launay, Pierre-yves Hatron
    Abstract:

    Objective: The objective of this study was to report the dramatic improvement of patients with systemic Capillary Leak Syndrome obtained with high-dose intravenous immunoglobulins. Design: Systemic Capillary Leak Syndrome is a rare and life-threatening 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. Because its cause is unknown, systemic Capillary Leak Syndrome treatment has remained largely supportive. Main results: Intravenous immunoglobulins administration to a patient with refractory systemic Capillary Leak Syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic Capillary Leak Syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic Capillary Leak Syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare. Conclusions: Intravenous immunoglobulins were effective against systemic Capillary Leak Syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation.

Eric Hachulla - One of the best experts on this subject based on the ideXlab platform.

  • systemic Capillary Leak Syndrome and autoimmune diseases a case series
    Seminars in Arthritis and Rheumatism, 2017
    Co-Authors: Aurelien Guffroy, Eric Hachulla, Benjamin Dervieux, Simon Gravier, C Martinez, Joelle Deibenerkaminsky, M Michel, Jeanchristophe Weber, Annesophie Korganow, Laurent Arnaud
    Abstract:

    Abstract Objectives Systemic Capillary Leak Syndrome (Clarkson's disease) is a rare entity characterized by recurrent and unpredictable attacks of Capillary Leakage of plasma fluid and proteins throughout the endothelium. Some cases are secondary. We describe the rare association between secondary Capillary Leak Syndrome (SCLS) and autoimmune diseases. Methods We conducted a nationwide, retrospective, observational, and collaborative study throughout the hospital units of the Club des Rhumatismes et Inflammations network (CRI) between March and August 2015. Inclusion criteria were patients with (1) Capillary Leakage episodes characterized by edema and elevated hematocrit, low albumin count without proteinuria, or other cause of protein loss; and (2) definite autoimmune diseases according to international classification criteria. Results The clinical and biological data of five patients (three women) were reviewed. Median age was 43.2 (17–55) years. Four patients had Sjogren Syndrome. One of them also fulfilled the criteria for systemic sclerosis ( n = 1). The fifth patient had polymyositis. During the 37.2 months of median follow-up (5.4–201), we recorded a total of 24 attacks, yielding an attack incidence of 91/100 patient-years. Laboratory tests revealed that three patients had anti-SSA/Ro antibodies. Only one patient had a monoclonal blood component (IgGκ). Three patients needed ICU support; one died during a flare. Conclusions We reported the first case series of a rare association of SCLS and autoimmune diseases, supporting the idea of some immune mediation in the pathogenesis of the former disease.

  • subcutaneous immunoglobulin therapy prevents systemic Capillary Leak Syndrome attack
    The American Journal of Medicine, 2016
    Co-Authors: Quentin Scanvion, Eric Hachulla, Pierre-yves Hatron, Guillaume Lefevre, Marc Lambert
    Abstract:

    We report the first case of systemic Capillary Leak Syndrome successfully treated with subcutaneous immunoglobulins.

  • myocardial involvement in systemic Capillary Leak Syndrome first demonstration by pathologic findings
    European heart journal. Acute cardiovascular care, 2012
    Co-Authors: Francis Juthier, Eric Hachulla, Pierre-yves Hatron, Pierre Vladimir Ennezat, Paul Fornes, Emmanuel Robin, Alain Prat, Brigitte Bouchindhomme, Carlo Banfi, Olivier Fouquet
    Abstract:

    This case vignette relates the unknown association between systemic Capillary Leak Syndrome, namely Clarkson’s Syndrome, and acute cardiac dysfunction. ‘Central extra-corporeal life support (ECLS)’ was needed for the management of an intractable cardiogenic shock. The acute cardiac condition completely resolved within few days. Pathology showed diffuse interstitial edema within the myocardium suggestive of cardiac involvement of the disease.

  • high dose intravenous immunoglobulins dramatically reverse systemic Capillary Leak Syndrome
    Critical Care Medicine, 2008
    Co-Authors: Marc Lambert, Eric Hachulla, S Morelldubois, Vincent Soland, Francois Fourrier, V. Queyrel, David Launay, Pierre-yves Hatron
    Abstract:

    Objective:The objective of this study was to report the dramatic improvement of patients with systemic Capillary Leak Syndrome obtained with high-dose intravenous immunoglobulins.Design:Systemic Capillary Leak Syndrome is a rare and life-threatening disorder characterized by hypotension that can lea

  • high dose intravenous immunoglobulins dramatically reverse systemic Capillary Leak Syndrome
    Critical Care Medicine, 2008
    Co-Authors: Marc Lambert, Eric Hachulla, S Morelldubois, Vincent Soland, Francois Fourrier, V. Queyrel, David Launay, Pierre-yves Hatron
    Abstract:

    Objective: The objective of this study was to report the dramatic improvement of patients with systemic Capillary Leak Syndrome obtained with high-dose intravenous immunoglobulins. Design: Systemic Capillary Leak Syndrome is a rare and life-threatening 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. Because its cause is unknown, systemic Capillary Leak Syndrome treatment has remained largely supportive. Main results: Intravenous immunoglobulins administration to a patient with refractory systemic Capillary Leak Syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic Capillary Leak Syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic Capillary Leak Syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare. Conclusions: Intravenous immunoglobulins were effective against systemic Capillary Leak Syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation.

J Di Paola - One of the best experts on this subject based on the ideXlab platform.

  • microangiopathic hemolytic anemia due to adamts 13 loss in idiopathic systemic Capillary Leak Syndrome
    Journal of Thrombosis and Haemostasis, 2016
    Co-Authors: Daniel C Moreira, Ralph Quinones, Xiayuan Liang, Dominic W Chung, J Di Paola
    Abstract:

    Essentials Idiopathic systemic Capillary Leak Syndrome (SCLS) is characterized by episodes of vascular Leakage. We present the case of a patient with SCLS who developed microangiopathic hemolytic anemia (MAHA). We propose that this anemia is the result of ADAMTS-13 loss in the third-space fluid. This suggests that MAHA can occur in patients with significant extravasation of proteins. Summary Idiopathic systemic Capillary Leak Syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular Leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this Syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patient developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia.

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