title: IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry.
abstract: 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.
authors.affiliation: From Université de la Méditerranée Aix-Marseille II (ME, LD, SB, LC, JS, AB,NJ, B. Granel, JB, PJW, GK, JRH, NS), Marseille; Service de Médecine Interne (ME, LC, VV, KM, EB, GK, JRH, NS), Service de Néphrologie (SB, NJ), and Laboratoire d'Immunologie (JB), Hôpital de la Conception, AssistancePublique-Hôpitaux de Marseille, Marseille; Service d'Anatomie Pathologique et de Neuropathologie (LD), and Service de Médecine Interne (JS, AB,PJW), Hôpital de la Timone, Assistance Publique-Hôpitaux de Marseille, Marseille; Service de Médecine Interne-Oncologie (MP), Hôpital D'instruction des Armées Desgenettes, Lyon; Service de Médecine Interne (PS), Hôtel-Dieu, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon; Service de Médecine Interne (MH), and Service de Rhumatologie (JMB), Hôtel Dieu, Centre Hospitalier Universitaire de Nantes, Nantes; Service de Médecine Interne(EA), Clinique Médicale B, Centre Hospitalier Universitaire de Strasbourg, Strasbourg; Inserm U897, Service de Médecine Interne et Tropicale (MLB), Hôpital Saint-André, Centre Hospitalier Universitaire de Bordeaux, Université Bordeaux 2 Victor-Segalen, Bordeaux; Service de Médecine Interne (MR), Hôtel Dieu, Centre Hospitalier Universitaire de Clermont-Ferrand; Service de Médecine Interne et Centre de Référence des Maladies auto-immunes et systémiques rares (JH), Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Paris 6, Paris; Service de Médecine Interne et Centre de référence des Cytopénies auto-immunes (B. Godeau), Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Université Paris 12, Créteil; Service de Médecine Interne (ABB), Centre Hospitalier Universitaire d'Angers, Angers; Service de Médecine Interne (TP), Hôpital Bichat-Claude Bernard, Assistance Publique- Hôpitaux de Paris, Université Paris 7, Paris; Service de Médecine Interne (JLP), Hôpital Robert Debré, Centre Hospitalier Universitaire de Reims, Reims; Service de Médecine Interne (CL, PR), and Service de Gastroentérologie(CS), Centre Hospitalier Universitaire la Miletrie, Poitiers; Service de Néphrologie (OM), Centre Hospitalier Universitaire de Nice, Nice; Service de Médecine Interne (B. Granel, FB), Hôpital Nord, Assistance Publique-Hôpitaux de Marseille, Marseille; Service de Médecine Interne (HR), Centre Hospitalier Lyon-Sud, Hospices Civils de Lyon, Pierre Benite; Laboratoire Neurobiologie des Interactions Cellulaires et Neurophysiopathologie (NICN) (JB), CNRS UMR 6184, Faculté de Médecine, Université Aix-Marseille, Marseille; Service de Néphrologie (JJB), Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie, Paris; INSERM, UMR-S 938, Hôpital Saint-Antoine (PA), Université Pierre et Marie Curie, Paris; and Centre d'Immunologie de Marseille-Luminy (NS), Université de la Méditerranée, case 906, Campus de Luminy, Marseille, France.
authors.forename: Mikael
authors.initials: M
authors.lastname: Ebbo
authors.forename: Laurent
authors.initials: L
authors.lastname: Daniel
authors.forename: Michel
authors.initials: M
authors.lastname: Pavic
authors.forename: Pascal
authors.initials: P
authors.lastname: Sève
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Andres
authors.forename: Stéphane
authors.initials: S
authors.lastname: Burtey
authors.forename: Laurent
authors.initials: L
authors.lastname: Chiche
authors.forename: Jacques
authors.initials: J
authors.lastname: Serratrice
authors.forename: Maïté
authors.initials: M
authors.lastname: Longy-Boursier
authors.forename: Marc
authors.initials: M
authors.lastname: Ruivard
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Anne-Bérengère
authors.initials: AB
authors.lastname: Beucher
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Berthelot
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Jean-Loup
authors.initials: JL
authors.lastname: Pennaforte
authors.forename: Audrey
authors.initials: A
authors.lastname: Benyamine
authors.forename: Noémie
authors.initials: N
authors.lastname: Jourde
authors.forename: Cédric
authors.initials: C
authors.lastname: Landron
authors.forename: Pascal
authors.initials: P
authors.lastname: Roblot
authors.forename: Olivier
authors.initials: O
authors.lastname: Moranne
authors.forename: Christine
authors.initials: C
authors.lastname: Silvain
authors.forename: Brigitte
authors.initials: B
authors.lastname: Granel
authors.forename: Fanny
authors.initials: F
authors.lastname: Bernard
authors.forename: Veronique
authors.initials: V
authors.lastname: Veit
authors.forename: Karin
authors.initials: K
authors.lastname: Mazodier
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Bernit
authors.forename: Hugues
authors.initials: H
authors.lastname: Rousset
authors.forename: José
authors.initials: J
authors.lastname: Boucraut
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Boffa
authors.forename: Pierre-Jean
authors.initials: PJ
authors.lastname: Weiller
authors.forename: Gilles
authors.initials: G
authors.lastname: Kaplanski
authors.forename: Pierre
authors.initials: P
authors.lastname: Aucouturier
authors.forename: Jean-Robert
authors.initials: JR
authors.lastname: Harlé
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D005602:France; D006801:Humans; D006942:Hypergammaglobulinemia / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012042:Registries; D012598:Sclerosis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents