Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease) - Immunologie-Immunopathologie-Immunothérapie Accéder directement au contenu
Article Dans Une Revue Journal of Allergy and Clinical Immunology: In Practice Année : 2022

Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease)

1 CHU Pitié-Salpêtrière [AP-HP]
2 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
3 CIMI - Centre d'Immunologie et des Maladies Infectieuses
4 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
5 CHU Angers - Centre Hospitalier Universitaire d'Angers
6 UPS/Inserm U1297 - I2MC - Institut des Maladies Métaboliques et Casdiovasculaires
7 CIC - CHU Bichat
8 LBBE - Laboratoire de Biométrie et Biologie Evolutive - UMR 5558
9 CHRU Montpellier - Hôpital Saint Eloi
10 I3 - Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière]
11 Centre hospitalier de Saint-Nazaire
12 Centre Hospitalier Henri Duffaut (Avignon)
13 CHU Estaing [Clermont-Ferrand]
14 Hôpital pasteur [Colmar]
15 UT3 - Université Toulouse III - Paul Sabatier
16 CHU Nantes - Centre Hospitalier Universitaire de Nantes
17 CHU Clermont-Ferrand
18 Hôpital Haut-Lévêque [CHU Bordeaux]
19 UNIROUEN - Université de Rouen Normandie
20 Centre de recherche du CHU Sainte-Justine / Research Center of the Sainte-Justine University Hospital [Montreal, Canada]
21 CHU - Hôpital Pasteur [Nice]
22 Hôpital Edouard Herriot [CHU - HCL]
23 UVSQ Santé - Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil
24 Service de médecine interne [CHU Ambroise Paré]
25 Hôpital Delafontaine
26 Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux)
27 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
Alexis Mathian
Franco Verlicchi
  • Fonction : Auteur
Antoine Dossier
  • Fonction : Auteur
Marie Josée Lucchini-Lecomte
  • Fonction : Auteur
Sylvie Le Moal
  • Fonction : Auteur
Béatrice Bibes
  • Fonction : Auteur
Giuseppe Malizia
  • Fonction : Auteur
Christian Agard
Martin J. Soubrier
Pierre Yves Lovey
  • Fonction : Auteur
Jorge Álvarez Troncoso
Avinash Aujayeb
  • Fonction : Auteur

Résumé

Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown. Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS. Methods: We conducted a retrospective multicenter study including all adult patients with monoclonal gammopathy–associated SCLS from the EurêClark registry who received at least 1 course of IVIG. The primary end point was overall survival according to IVIG withdrawal. Results: Fifty-nine patients of mean ± SD age 51 ± 13 years were included. Overall cumulative probabilities of 2-, 5-, 10- and 15-year survival were 100%, 85%, 72%, 44%, respectively. The IVIG was withdrawn at least once in 18 patients (31%; W+ group) and never in 41 patients (69%; W– group). Cumulative probabilities of 10-year survival in W+ versus W– groups were 50% and 83% (log rank test, P = .02), respectively. Relapse rate and the median number of relapses in the W+ versus the W– groups were 72% versus 58% (P = 0.3) and 2.5 (0.3–4) versus 1 (0–2) (P = .03), respectively. The IVIG tapering was not statistically associated with increased person-year incidence of attacks using a mixed linear model. Conclusions: The IVIG withdrawal was associated with increased mortality and higher rate of recurrence in SCLS patients. The IVIG tapering might be cautiously considered in stable SCLS patients.

Dates et versions

hal-03888981 , version 1 (07-12-2022)

Identifiants

Citer

Quentin Moyon, Marc Pineton de Chambrun, Marie Gousseff, Alexis Mathian, Miguel Hie, et al.. Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease). Journal of Allergy and Clinical Immunology: In Practice, 2022, 10 (11), pp.2889-2895. ⟨10.1016/j.jaip.2022.07.006⟩. ⟨hal-03888981⟩
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