IgG4-Related Systemic Disease Features and Treatment Response in a French Cohort: Results of a Multicenter Registry

Mikael Ebbo; Laurent Daniel; Michel Pavic; Pascal Seve; Mohamed Hamidou; Emmanuel Andres; Stephane Burtey; Laurent Chiche; Jacques Serratrice; Maite Longy-Boursier; Marc Ruivard; Julien Haroche; Bertrand Godeau; Anne-Berengere Beucher; Jean-Marie Berthelot; Thomas Papo; Jean-Loup Pennaforte; Audrey Benyamine; Noemie Jourde; Cedric Landron; Pascal Roblot; Olivier Moranne; Christine Silvain; Brigitte Granel; Fanny Bernard; Veronique Veit; Karin Mazodier; Emmanuelle Bernit; Hugues Rousset; Jose Boucraut; Jean-Jacques Boffa; Pierre-Jean Weiller; Gilles Kaplanski; Pierre Aucouturier; Jean-Robert Harle; Nicolas Schleinitz

doi:10.1097/MD.0b013e3182433d77

Retour

IgG4-Related Systemic Disease Features and Treatment Response in a French Cohort: Results of a Multicenter Registry

Article de revue

Avec comité de lecture

IgG4-Related Systemic Disease Features and Treatment Response in a French Cohort: Results of a Multicenter Registry

Mikael Ebbo, Laurent Daniel, Michel Pavic, Pascal Seve, Mohamed Hamidou, Emmanuel Andres, Stephane Burtey, Laurent Chiche, Jacques Serratrice, Maite Longy-Boursier, …

Medicine (Baltimore), Vol.91(1), pp.49-56

01/01/2012

DOI: https://doi.org/10.1097/MD.0b013e3182433d77

PMID: 22198501

Résumé

General & Internal Medicine

Life Sciences & Biomedicine

Medicine, General & Internal

Science & Technology

IgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.

Indicateurs

1 Consultations de la notice

Détails

Titre: IgG4-Related Systemic Disease Features and Treatment Response in a French Cohort: Results of a Multicenter Registry
Créateurs - sans rôle: Mikael Ebbo - Univ Mediterrane Aix Marseille II, Marseille, France
Laurent Daniel - Univ Mediterrane Aix Marseille II, Marseille, France
Michel Pavic - Hop Instruct Armees Desgenettes, Serv Med Interne Oncol, Lyon, France
Pascal Seve - Univ Lyon 1, Serv Med Interne, Hotel Dieu, Hosp Civils Lyon, Lyon, France
Mohamed Hamidou - Nantes Université
Emmanuel Andres - Univ Strasbourg, Serv Med Interne, Clin Med B, Ctr Hosp, Strasbourg, France
Stephane Burtey - Univ Mediterrane Aix Marseille II, Marseille, France
Laurent Chiche - Univ Mediterrane Aix Marseille II, Marseille, France
Jacques Serratrice - Univ Mediterrane Aix Marseille II, Marseille, France
Maite Longy-Boursier - Univ Bordeaux 2, INSERM, Serv Med Interne & Trop, Hop St Andre,CHU Bordeaux,U897, F-33076 Bordeaux, France
Marc Ruivard - Ctr Hosp Univ Clermont Ferrand, Serv Med Interne, Hotel Dieu, Clermont Ferrand, France
Julien Haroche - Univ Paris 06, Serv Med Interne, Paris, France
Bertrand Godeau - Univ Paris 12, Serv Med Interne, Creteil, France
Anne-Berengere Beucher - CHU Angers, Serv Med Interne, Angers, France
Jean-Marie Berthelot - Univ Nantes, Serv Rhumatol, Hotel Dieu, Ctr Hosp, Nantes, France
Thomas Papo - Univ Paris 07, Serv Med Interne, Hop Bichat Claude Bernard, AP HP, Paris, France
Jean-Loup Pennaforte - Ctr Hosp Univ Reims, Serv Med Interne, Hop Robert Debre, Reims, France
Audrey Benyamine - Univ Mediterrane Aix Marseille II, Marseille, France
Noemie Jourde - Univ Mediterrane Aix Marseille II, Marseille, France
Cedric Landron - CHU La Miletrie, Serv Med Interne, F-86021 Poitiers, France
Pascal Roblot - CHU La Miletrie, Serv Med Interne, F-86021 Poitiers, France
Olivier Moranne - Ctr Hosp Univ Nice, Serv Nephrol, Nice, France
Christine Silvain - CHU La Miletrie, Gastroenterol Serv, F-86021 Poitiers, France
Brigitte Granel - Univ Mediterrane Aix Marseille II, Marseille, France
Fanny Bernard - Hop Nord Marseille, Assistance Publ Hop Marseille, Serv Med Interne, Marseille, France
Veronique Veit - Hop Conception, Assistance Publ Hop Marseille, Serv Med Interne, Marseille, France
Karin Mazodier - Hop Conception, Assistance Publ Hop Marseille, Serv Med Interne, Marseille, France
Emmanuelle Bernit - Hop Conception, Assistance Publ Hop Marseille, Serv Med Interne, Marseille, France
Hugues Rousset - Hosp Civils, Serv Med Interne, Ctr Hosp Lyon Sud, Pierre Benite, France
Jose Boucraut - Univ Mediterrane Aix Marseille II, Marseille, France
Jean-Jacques Boffa - Univ Paris 06, Serv Nephrol, Hop Tenon, AP HP, Paris, France
Pierre-Jean Weiller - Univ Mediterrane Aix Marseille II, Marseille, France
Gilles Kaplanski - Univ Mediterrane Aix Marseille II, Marseille, France
Pierre Aucouturier - Univ Paris 06, INSERM, Hop St Antoine, UMR S 938, Paris, France
Jean-Robert Harle - Univ Mediterrane Aix Marseille II, Marseille, France
Nicolas Schleinitz - Univ Mediterrane Aix Marseille II, Marseille, France
Détails de publication: Medicine (Baltimore), Vol.91(1), pp.49-56
Éditeur: Lippincott Williams & Wilkins
Nombre de pages: 8
Identifiants: 99150142709311
Unité académique: Institut Desbrest d Epidemiologie et de Sante Publique - IDESP
Langue: English
Type de ressource: Journal article