Hépato-Gastro & Oncologie Digestive


La maladie associée aux IgG4 Volume 30, issue 7, September 2023


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CHU Timone , Service de Médecine Interne, 264 rue Saint Pierre, 13005 Marseille
* Correspondance : N. Schleinitz

IgG4 related disease (IgG4-RD) is a rare and recently described fibro-inflammatory disease. IgG4-RD has protean manifestations since nearly every organ can be affected as a consequence of inflammatory infiltrate and subsequent fibrosis. Diagnosis requires a set of clinical, biological and histological features followed by an exclusion of differential diagnosis (even in the setting of IgG4 elevation and/or compatible histology). The new ACR/EULAR (American College of Rheumatology/European Alliance of Associations for Rheumatology) classification criteria reflects the steps of reasoning and the complexity of an IgG4-RD diagnosis. The disease is characterized by an excellent response to corticosteroids and immunosuppressive therapies. Main complications are secondary to organ dysfunction, frequent relapse and side effects from treatments.