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Published on: February 8, 2019
IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry
Mikael Ebbo1, Laurent Daniel, Michel Pavic
1From 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.
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.
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