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Journal Articles The American Journal of Medicine Year : 2017

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

Marc Pineton de Chambrun (1) , Marie Gousseff (2) , Wladimir Mauhin (3) , Jean-Christophe Lega (4) , Marc Lambert (5) , Sophie Rivière (6) , Antoine Dossier (7) , Marc Ruivard (8) , François Lhote (9) , Gilles Blaison (10) , Laurent Alric (11) , Christian Agard (12) , David Saadoun (13) , Julie Graveleau (14) , Martin Soubrier (15) , Marie-Josée Lucchini-Lecomte (16) , Christine Christides (17) , Annick Bosseray (18) , Hervé Lévesque (19) , François Viallard (20) , Nathalie Tieulie (21) , Pierre-Yves Lovey (22) , Sylvie Le Moal (23) , Béatrice Bibes (24) , Giuseppe Malizia , Pierre Abgueguen (25) , François Liferman (26) , Jacques Ninet (27) , Pierre-Yves Hatron (28) , Zahir Amoura (29) , Arnaud Hot (27) , Laurent Argaud (30) , Romain Hernu , Sylvie de La Salle , Stanislas Ledochowski , Sophie Moreau (31) , Thomas Papo (32) , Romain Sonneville (33) , Bruno Verdière , Sybille Merceron (34) , Nathalie Zappella , Mickael Landais , Nicolas Limal , Damien Contou (35) , Thomas Similowski (36) , Alexandre Demoule (37) , Bertrand Souweine (38) , Julien Haroche (36) , Julien Boileau (39) , Bernard Lecomte (40) , Thomas Hanslik (41) , Antoine Vieillard-Baron (42) , Nicolas Terzi (43) , Caroline Bulte , Aline Talasczka , Eric Hachulla (5) , Olivier Decaux (44) , Florent Ibouanga , Bertrand Arnulf (45) , Matthieu Groh (46) , Elie Azoulay (47) , Marc Benedit , Assaad Maalouf , Bruno Moulin (48) , Fleur Cohen-Aubart (36) , Raymond Friolet , Micheline Pha , Georges-Etienne Rivard (49) , Eric Rondeau , Philippe Debourdeau (50) , Marc Puidupin , Francois Beloncle (51) , Jérôme Devaquet (52) , Claire Presne (53) , Jean-Marc Mazou , Maude Andrieu , Sylvie Paulus , Yannick Fédun , Jean-Paul Mira (54) , Jean-Herlé Raphalen , Oscar Len Abad , Hervé Devilliers (55) , Alister Rogers , Pascal Godmer (56) , Charles-Edouard Luyt (57) , Alain Combes (57) , Miguel Hie (36) , Alexis Mathian (7)
1 CHU Tenon [AP-HP]
2 Institut Pasteur de Madagascar
3 Centre de recherche en Myologie – U974 SU-INSERM
4 HCL - Hospices Civils de Lyon
5 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
6 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
7 CIMI - Centre d'Immunologie et de Maladies Infectieuses
8 IP - Institut Pascal
9 CNRS - Centre National de la Recherche Scientifique
10 Department of Internal Medicine
11 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
12 Service de médecine interne [Nantes]
13 I3 - Immunologie - Immunopathologie - Immunothérapeutique
14 Centre hospitalier de Saint-Nazaire
15 CHU Clermont-Ferrand
16 Centre hospitalier d'Ajaccio
17 Centre Hospitalier Henri Duffaut (Avignon)
18 Clinique de médecine interne
19 Service de Médecine Interne [CHU Rouen]
20 IHU-LIRYC
21 Hôpital l'Archet
22 Centre Hospitalier du Valais Romand [Sion, Switzerland]
23 CH Saint-Brieuc - Centre hospitalier de Saint-Brieuc [Hôpital Yves Le Foll]
24 CHP Saint Grégoire
25 Service des maladies infectieuses et tropicales [CHU Angers]
26 MEDECINE INTERNE - Médecine Interne Dax
27 Service de Médecine Interne
28 Service de médecine interne [Lille]
29 Immunité et Infection
30 Hôpital Edouard Herriot [CHU - HCL]
31 ACP - Analyse Comparée des Pouvoirs
32 Department of Internal Medicine
33 Hôpital Bichat - Claude Bernard
34 Service de soins intensifs
35 Service de réanimation médicale
36 CHU Pitié-Salpêtrière [AP-HP]
37 Neurophysiologie Respiratoire Expérimentale et Clinique
38 LMGE - Laboratoire Microorganismes : Génome et Environnement
39 Department of Clinical Immunology
40 Médecine générale
41 Service de Médecine Interne
42 Service de réanimation medico-chirurgicale [CHU Raymond-Poincaré]
43 Service de réanimation médicale [CHU Caen]
44 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
45 Service d'hématologie biologique
46 Service des maladies infectieuses et tropicales
47 Medical ICU
48 Service de néphrologie
49 Service d'Hématologie-Oncologie
50 Dep Oncol - AVIGNON - Département d'Oncologie
51 Service de Réanimation Médicale et de Médecine Hyperbare [Angers]
52 Service d'anesthésie-réanimation SAMU94-SMUR94 [Mondor]
53 Service de Néphrologie - Médecine Interne
54 UMR_S567 / UMR 8104 - Institut Cochin
55 Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon]
56 Service de médecine interne [CHU Bretagnes Atlantique]
57 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
Jean-Christophe Lega
Marc Ruivard
  • Function : Author
  • PersonId : 1024558
Laurent Alric
  • Function : Author
  • PersonId : 881462
Christian Agard
Martin Soubrier
François Viallard
  • Function : Author
Giuseppe Malizia
  • Function : Author
Romain Hernu
  • Function : Author
Sylvie de La Salle
  • Function : Author
Stanislas Ledochowski
  • Function : Author
Sophie Moreau
Romain Sonneville
  • Function : Author
  • PersonId : 911537
Bruno Verdière
  • Function : Author
Sybille Merceron
  • Function : Author
  • PersonId : 944493
Nathalie Zappella
  • Function : Author
Mickael Landais
Nicolas Limal
  • Function : Author
Thomas Similowski
Nicolas Terzi
Caroline Bulte
  • Function : Author
Aline Talasczka
  • Function : Author
Florent Ibouanga
  • Function : Author
Elie Azoulay
Marc Benedit
  • Function : Author
Assaad Maalouf
  • Function : Author
Fleur Cohen-Aubart
  • Function : Author
  • PersonId : 1002089
Raymond Friolet
  • Function : Author
Micheline Pha
  • Function : Author
Eric Rondeau
  • Function : Author
  • PersonId : 835505
Marc Puidupin
  • Function : Author
Jean-Marc Mazou
  • Function : Author
Maude Andrieu
  • Function : Author
Sylvie Paulus
  • Function : Author
Yannick Fédun
  • Function : Author
Jean-Paul Mira
  • Function : Author
  • PersonId : 928244
Jean-Herlé Raphalen
  • Function : Author
Oscar Len Abad
  • Function : Author
Alister Rogers
  • Function : Author

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.

Dates and versions

hal-01656768 , version 1 (06-12-2017)

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Marc Pineton de Chambrun, Marie Gousseff, Wladimir Mauhin, Jean-Christophe Lega, Marc Lambert, et al.. Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. The American Journal of Medicine, 2017, 130 (10), pp.1219.e19 - 1219.e27. ⟨10.1016/j.amjmed.2017.05.023⟩. ⟨hal-01656768⟩
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