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 Institut E3M - Endocrinologie, Maladies Métaboliques et Médecine interne [Hôpital Universitaire Pitié-Salpêtrière]
2 CRMR - Centre de référence des maladies auto-immunes et auto-inflammatoires systémiques rares de l'adulte d'Île de France, Est et Ouest / Reference center for rare systemic autoimmune and autoinflammatory diseases in adults in Île-de-France, East and West
3 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
4 CIMI - Centre d'Immunologie et des Maladies Infectieuses
5 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
6 Service de médecine interne et immunologie Clinique [Angers]
7 UPS/Inserm U1297 - I2MC - Institut des Maladies Métaboliques et Casdiovasculaires
8 CIC - CHU Bichat
9 LBBE - Laboratoire de Biométrie et Biologie Evolutive - UMR 5558
10 Hôpital Saint Eloi [CHU Montpellier]
11 I3 - Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière]
12 Centre hospitalier de Saint-Nazaire
13 CH Avignon - Centre Hospitalier Henri Duffaut [Avignon]
14 CHU Estaing [Clermont-Ferrand]
15 Hôpital pasteur [Colmar]
16 UT3 - Université Toulouse III - Paul Sabatier
17 Service Médecine interne [CHU Toulouse]
18 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
19 CHU Clermont-Ferrand
20 Hôpital Haut-Lévêque [CHU Bordeaux]
21 UNIROUEN - Université de Rouen Normandie
22 Centre de recherche du CHU Sainte-Justine / Research Center of the Sainte-Justine University Hospital [Montreal, Canada]
23 CHU - Hôpital Pasteur [Nice]
24 Hôpital Edouard Herriot [CHU - HCL]
25 UVSQ Santé - UFR Sciences de la santé Simone Veil
26 Service de médecine interne [CHU Ambroise Paré]
27 Hôpital Delafontaine
28 CH Eure-Seine - Centre Hospitalier Eure-Seine - Hôpital d'Evreux et de Vernon
29 CHU Pitié-Salpêtrière [AP-HP]
30 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
Franco Verlicchi
  • Fonction : Auteur
Marie Josée Lucchini-Lecomte
  • Fonction : Auteur
Sylvie Le Moal
  • Fonction : Auteur
Béatrice Bibes
  • Fonction : Auteur
Giuseppe Malizia
  • Fonction : Auteur
Martin J. Soubrier
Pierre Yves Lovey
  • Fonction : Auteur
Jorge Álvarez Troncoso
Avinash Aujayeb
  • Fonction : Auteur
Zahir Amoura

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)

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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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