Academic Journal
French protocol for diagnosis and management (guidelines) of IgG4-related disease.
| Τίτλος: | French protocol for diagnosis and management (guidelines) of IgG4-related disease. |
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| Συγγραφείς: | Schleinitz N; Département de Médecine Interne, Timone Hospital, Assistance publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France., Audia S; Université Bourgogne Europe, Centre Hospitalier Universitaire Dijon-Bourgogne, Service de Médecine Interne et Immunologie Clinique, Dijon, France., Cohen F; Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre for Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France., Galicier L; Département de Médecine Interne, Timone Hospital, Assistance publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France., Groh M; Department of Internal Medicine, Clinical Immunology and Hematology, National Reference Centre for Hypereosinophilic Syndromes (CEREO), Foch Hospital, Suresnes, France; Université de Versailles Saint-Quentin-en-Yvelines, Montigny-le-Bretonneux, France., Hachulla E; Department of Internal Medicine and Clinical Immunology, National Reference Centre for Rare Systemic Auto-immune and Auto-inflammatory Diseases, Inserm, CHU Lille, U1286-INFINITE, University of Lille, Lille, France., Neel A; Internal Medicine Department, Hôtel-Dieu, Nantes University Hospital, Nantes, France., Rebours V; Université de Paris Cité, Paris, France; Department of Pancreatology and Digestive Oncology, DMU DIGEST, Beaujon Hospital, AP-HP, Clichy, Inserm UMR1149, National Reference Centre for Pancreatic Rare Diseases (PaRaDis), France., Seve P; Department of Internal Medicine, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université Claude-Bernard Lyon 1, Lyon, France; Research on Healthcare Performance (RESHAPE), Inserm U1290, Lyon, France., Viallard JF; Department of Internal Medicine and Infectious Diseases, Haut-Lévêque Hospital, University Hospital of Bordeaux, Pessac Cedex, France; Inserm U1034, Bordeaux University, Pessac Cedex, France., Zaidan M; Department of Nephrology, Hôpital Bicêtre, Assistance publique-Hôpitaux de Paris, Université Paris-Saclay, Le Kremlin-Bicêtre, France., Ebbo M; Département de Médecine Interne, Timone Hospital, Assistance publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France. Electronic address: mikael.ebbo@ap-hm.fr. |
| Συλλογικό Έργο: | Collaborators |
| Πηγή: | La Revue de medecine interne [Rev Med Interne] 2026 Jul; Vol. 47 (7), pp. 362-383. Date of Electronic Publication: 2026 Jun 11. |
| Τύπος έκδοσης: | Journal Article; Practice Guideline; Review |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Elsevier Country of Publication: France NLM ID: 8101383 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1768-3122 (Electronic) Linking ISSN: 02488663 NLM ISO Abbreviation: Rev Med Interne Subsets: MEDLINE |
| Imprint Name(s): | Publication: Paris : Elsevier Original Publication: Paris : Baillière, [1980- |
| Ιατρικοί όροι (MeSH): | Immunoglobulin G4-Related Disease*/diagnosis , Immunoglobulin G4-Related Disease*/therapy , Immunoglobulin G*/blood , Clinical Protocols*/standards , Practice Guidelines as Topic*, France/epidemiology ; Glucocorticoids/therapeutic use ; Humans ; Disease Management |
| Περίληψη: | IgG4-related disease (IgG4-RD) is a recently described entity comprising pseudo-tumoral and inflammatory conditions that were previously considered separately. It is characterised by specific histological abnormalities, including polyclonal lymphoplasmacytic infiltration, fibrosis and contingent of IgG4+ plasma cells, often associated with elevated serum IgG4 levels and a good response to glucocorticoids. The clinical presentation is highly heterogeneous and requires a multidisciplinary assessment. The diagnosis is based on the identification of suggestive clinical or radiological signs, such as pancreatic, salivary or lacrimal gland involvement, retroperitoneal fibrosis, cholangitis, aortitis, lymphadenopathy or interstitial nephritis. It is also based on the search for laboratory evidence of abnormalities such as polyclonal gammopathy, elevated serum IgG4 levels and complement consumption, and is most often confirmed by organ biopsy. The disease progresses slowly, with gradual symptoms and a risk of sequelae related to fibrosis, particularly pancreatic or renal failure. Management is based on providing the patient with comprehensive information about symptoms, warning signs, adverse events of treatments, vaccination, diet and physical activity. Initial treatment consists of oral glucocorticoids at a dose of 0.4 to 0.6mg/kg/day for two to four weeks, followed by a gradual tapering until discontinuation at three months if possible. This treatment induces remission in more than 90% of cases, but relapses are common, sometimes requiring treatment with immunosuppressants. Follow-up includes regular consultations, imaging tests and laboratory workups, in particular serum IgG4 measurement, in order to monitor disease activity, predict relapses and prevent complications. (Copyright © 2026 The Authors. Published by Elsevier Masson SAS.. All rights reserved.) |
| Competing Interests: | Disclosure of interest All participants in the development of the FPDM completed a declaration of interest. The declarations of interest are online and available on the FAI(2)R website. |
| Contributed Indexing: | Investigator: S Abad, Internal Medicine, Avicenne, France.; B Bader-Meunier, Paediatric Rheumatology, Necker, Paris, France.; M Chartier, General Medicine, Marseille, France.; V Cottin, Pulmonology, Lyon, France.; L Daniel, Anatomical Pathology, Marseille, France.; R Gimenez, French IgG4-RD Patient Association, France.; JE Kahn, Internal Medicine, Boulogne, France.; G Lefevre, Internal Medicine, Lille, France.; V Meignin, Anatomical Pathology, Saint Louis, Paris, France.; A Perlat, Internal Medicine, Rennes, France.; G Pugnet, Internal Medicine, Toulouse, France.; M Puyade, Internal Medicine, Poitiers, France.; K Sacre, Internal Medicine, Bichat, Paris, France.; M Samson, Internal Medicine, Dijon, France.; B Terrier, Internal Medicine, Cochin, Paris, France. Keywords: Diagnosis; Diagnostic; IgG4-related disease; Maladie associée aux IgG4; Management; Prise en charge |
| Substance Nomenclature: | 0 (Immunoglobulin G) 0 (Glucocorticoids) |
| Entry Date(s): | Date Created: 20260611 Date Completed: 20260730 Latest Revision: 20260730 |
| Update Code: | 20260731 |
| DOI: | 10.1016/j.revmed.2026.05.013 |
| PMID: | 42276880 |
| Βάση Δεδομένων: | MEDLINE |
| ISSN: | 1768-3122 |
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| DOI: | 10.1016/j.revmed.2026.05.013 |