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1. The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission.

Author

Mauhin, Wladimir, Amoura, Zahir, de Chambrun, Marc Pineton, Verdière, Bruno, Merceron, Sybille, Zappella, Nathalie, Landais, Mickael, Contou, Damien, Demoule, Alexandre, Paulus, Sylvie, Souweine, Bertrand, Lecomte, Bernard, Vieillard-Baron, Antoine, Terzi, Nicolas, Luyt, Charles-Edouard, Combes, Alain, Azoulay, Elie, Friolet, Raymond, Puidupin, Marc, and Devaquet, Jérôme

Subjects
*CAPILLARY leak syndrome, *HYPOVOLEMIC anemia, *INTENSIVE care patients, *CRITICAL care medicine, *MONOCLONAL gammopathies, *HOSPITAL admission & discharge
Abstract

Objective: Systemic capillary-leak syndrome is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission. This study was undertaken to describe the clinical pictures and ICU management of severe systemic capillary-leak syndrome episodes. Design, Setting, Patients: This multicenter retrospective analysis concerned patients entered in the European Clarkson's disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016. Measurements and Main Results: Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± sd age, 51 ± 11.4 yr) were included. Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains. ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9- 22 g/dL) and 50 g/L (36.5-58.5 g/L). IV immunoglobulins were infused (IV immunoglobulin) during 15 episodes (25.4%). A compartment syndrome developed during 12 episodes (20.3%). Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients' last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2-140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6-180]; p = 0.02) as independent predictors of hospital mortality. Conclusions: We described the largest cohort of severe systemic capillary-leak syndrome flares requiring ICU admission. High-volume fluid therapy was independently associated with poorer outcomes. IV immunoglobulin use was not associated with improved survival; hence, their use should be considered prudently and needs further evaluation in future studies. [ABSTRACT FROM AUTHOR]

Published
2017
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2. The consequences of COVID-19 pandemic on patients with monoclonal gammopathy-associated systemic capillary leak syndrome (Clarkson disease)

Author

Marc Pineton de Chambrun, Quentin Moyon, Stanislas Faguer, Geoffrey Urbanski, Alexis Mathian, Noémie Zucman, Marie Werner, Charles-Edouard Luyt, Franco Verlicchi, Zahir Amoura, Marie Gousseff, Wladimir Mauhin, Arnaud Hot, Jean-Christophe Lega, Marc Lambert, Sophie Riviere, Antoine Dossier, Marc Ruivard, François Lhote, Gilles Blaison, Sybille Merceron, Nathalie Zapella, Laurent Alric, Christian Agard, Mathieu Lacout, David Saadoun, Julie Graveleau, Martin Soubrier, Julien Haroche, Julien Boileau, Marie-Josee Lucchini-Lecomte, Thomas Hanslik, Christine Christides, Hervé Levesque, Aline Talasczka, Caroline Bulte, Eric Hachulla, Olivier Decaux, Romain Sonneville, Florent Ibouanga, Bertrand Arnulf, Marcel Benedit, Jean François Viallard, Nathalie Tieulie, Fadi Haddad, Bruno Moulin, Fleur Cohen-Aubert, Pierre-Yves Lovey, Sylvie le Moal, Béatrice Bibes, Georges-Etienne Rivard, Eric Rondeau, Giuseppe Malizia, Philippe Debourdeau, Pierre Abgueguen, Annick Bosseray, Jérôme Devaquet, Claire Presne, François Liferman, Nicolas Limal, Laurent Argaud, Romain Hernu, Sylvie de la Salle, Jorge Álvarez Troncoso, John Harty, Pascal Godmer, Miguel Hie, Thomas Papo, and Pierre-Yves Hatron

Subjects
SARS-CoV-2, systemic capillary-leak syndrome, Paraproteinemias, Immunology and Allergy, COVID-19, Humans, Clinical Communications, Clarkson’s disease, vaccination, Pandemics, Capillary Leak Syndrome
Published
2021

3. Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome

Author

Marc Pineton de Chambrun, Marie Gousseff, Wladimir Mauhin, Jean-Christophe Lega, Marc Lambert, Sophie Rivière, Antoine Dossier, Marc Ruivard, François Lhote, Gilles Blaison, Laurent Alric, Christian Agard, David Saadoun, Julie Graveleau, Martin Soubrier, Marie-Josée Lucchini-Lecomte, Christine Christides, Annick Bosseray, Hervé Levesque, Jean-François Viallard, Nathalie Tieulie, Pierre-Yves Lovey, Sylvie Le Moal, Béatrice Bibes, Giuseppe Malizia, Pierre Abgueguen, François Lifermann, Jacques Ninet, Pierre-Yves Hatron, Zahir Amoura, Arnaud Hot, Laurent Argaud, Romain Hernu, Sylvie de la Salle, Stanislas Ledochowski, Anne-Sophie Moreau, Thomas Papo, Romain Sonneville, Bruno Verdière, Sybille Merceron, Nathalie Zappella, Mickael Landais, Nicolas Limal, Damien Contou, Thomas Similowski, Alexandre Demoule, Bertrand Souweine, Julien Haroche, Julien Boileau, Bernard Lecomte, Thomas Hanslik, Antoine Vieillard-Baron, Nicolas Terzi, Caroline Bulte, Aline Talasczka, Eric Hachulla, Olivier Decaux, Florent Ibouanga, Bertrand Arnulf, Matthieu Groh, Elie Azoulay, Marcel Benedit, Assaad Maalouf, Bruno Moulin, Fleur Cohen-Aubart, Raymond Friolet, Sylvie le Moal, Micheline Pha, Georges-Etienne Rivard, Eric Rondeau, Philippe Debourdeau, Marc Puidupin, François Beloncle, Jérôme Devaquet, Claire Presne, François Liferman, Jean-Marc Mazou, Maude Andrieu, Sylvie Paulus, Yannick Fedun, Jean-Paul Mira, Jean-Herlé Raphalen, Oscar Len Abad, Hervé Devilliers, Alister Rogers, Pascal Godmer, Charles-Edouard Luyt, Alain Combes, Miguel Hie, Alexis Mathian, CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Pasteur de Madagascar, Réseau International des Instituts Pasteur (RIIP), Centre de recherche en myologie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Centre d'Immunologie et de Maladies Infectieuses (CIMI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut Pascal (IP), SIGMA Clermont (SIGMA Clermont)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Centre National de la Recherche Scientifique (CNRS), Centre National de la Recherche Scientifique (CNRS), Department of Internal Medicine, Hôpital pasteur [Colmar], CHU Toulouse [Toulouse], Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Immunologie - Immunopathologie - Immunothérapeutique (I3), Centre hospitalier de Saint-Nazaire, CHU Clermont-Ferrand, Centre hospitalier d'Ajaccio, Centre Hospitalier Henri Duffaut (Avignon), Clinique de médecine interne, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), IHU-LIRYC, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux], Hôpital l'Archet, Centre Hospitalier du Valais Romand [Sion, Switzerland], CHU de Saint-Brieuc, CHP Saint Grégoire, Service des maladies infectieuses et tropicales [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Médecine Interne Dax (MEDECINE INTERNE), Hopital, Service de Médecine Interne, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de médecine interne [Lille], Immunité et Infection, Université Pierre et Marie Curie - Paris 6 (UPMC)-IFR113-Institut National de la Santé et de la Recherche Médicale (INSERM), Analyse Comparée des Pouvoirs (ACP), Université Paris-Est Marne-la-Vallée (UPEM), Université Paris Diderot - Paris 7 (UPD7), Hôpital Bichat - Claude Bernard, Service de soins intensifs, Centre Hospitalier de Versailles André Mignot (CHV), Service de réanimation médicale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de Pneumologie et Réanimation Médicale [CHU Pitié-Salpêtrière] (Département ' R3S '), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Neurophysiologie Respiratoire Expérimentale et Clinique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire Microorganismes : Génome et Environnement (LMGE), Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Université d'Auvergne - Clermont-Ferrand I (UdA)-Centre National de la Recherche Scientifique (CNRS), Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Department of Clinical Immunology, CHU Strasbourg, Médecine générale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Ambroise Paré [AP-HP], Service de réanimation medico-chirurgicale [CHU Raymond-Poincaré], Service de réanimation médicale [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Pontchaillou [Rennes], Service d'hématologie biologique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service des maladies infectieuses et tropicales, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Medical ICU, Service de néphrologie, Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), Service d'Hématologie-Oncologie, Hôpital Ste-Justine, Département d'Oncologie (Dep Oncol - AVIGNON), Institut Ste Catherine, Service de Réanimation Médicale et de Médecine Hyperbare [Angers], Service d'anesthésie-réanimation SAMU94-SMUR94 [Mondor], Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-hôpital Sud, Institut Cochin (UMR_S567 / UMR 8104), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de médecine interne [CHU Bretagnes Atlantique], CHU Bretagnes Atlantique, Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), SIGMA Clermont (SIGMA Clermont)-Centre National de la Recherche Scientifique (CNRS)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), Service de Medecine Interne, CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], Pharmacochimie et Biologie pour le Développement (PHARMA-DEV), Institut de Recherche pour le Développement (IRD)-Institut de Chimie de Toulouse (ICT-FR 2599), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie du CNRS (INC)-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie du CNRS (INC), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Centre National de la Recherche Scientifique (CNRS)-Université d'Auvergne - Clermont-Ferrand I (UdA), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Université de Caen Normandie (UNICAEN), Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), and Service de médecine interne et d'immunologie clinique [CHU Pitié-Salpêtrière]

Subjects
Male, medicine.medical_specialty, Paraproteinemias, Context (language use), Intravenous immunoglobulins, Monoclonal gammopathy-associated systemic capillary-leak syndrome, Systemic capillary-leak syndrome, 030204 cardiovascular system & hematology, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Theophylline, Interquartile range, Internal medicine, medicine, Terbutaline, Systemic capillary leak syndrome, Humans, 030212 general & internal medicine, Multiple myeloma, Clarkson disease, business.industry, Hazard ratio, Immunoglobulins, Intravenous, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, General Medicine, Middle Aged, medicine.disease, Survival Analysis, 3. Good health, Surgery, Cohort, Monoclonal, Female, business, Capillary Leak Syndrome, Cohort study
Abstract

International audience; 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 (EurêClark) 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.

Published
2016

4. Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome.

Author

Pineton de Chambrun, Marc, Gousseff, Marie, Mauhin, Wladimir, Lega, Jean-Christophe, Lambert, Marc, Rivière, Sophie, Dossier, Antoine, Ruivard, Marc, Lhote, François, Blaison, Gilles, Alric, Laurent, Agard, Christian, Saadoun, David, Graveleau, Julie, Soubrier, Martin, Lucchini-Lecomte, Marie-Josée, Christides, Christine, Bosseray, Annick, Levesque, Hervé, and Viallard, Jean-François

Subjects
*CAPILLARY leak syndrome, *MONOCLONAL gammopathies, *INTRAVENOUS immunoglobulins, *IMMUNOGLOBULIN G, *THEOPHYLLINE, *THERAPEUTIC use of immunoglobulins, *TERBUTALINE, *PARAPROTEINEMIA, *SURVIVAL analysis (Biometry), *DISEASE complications, *THERAPEUTICS
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 (EurêClark) 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. [ABSTRACT FROM AUTHOR]

Published
2017
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