Therapy recommendations for IgG4-related disease

IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory rheumatic disease. Its treatment is initially based on clinical experience - due to the limited studies and lack of approved medications. Nevertheless, the evidence base is growing over time. After clinical assessment of the necessity for systemic therapy, symptomatic patients typically undergo remission induction with oral glucocorticoid therapy. Remission maintenance is achieved through gradual dose reduction, which initially aims for a maintenance dose of 2.5 to 7.5 mg/day. In cases of frequent relapses or unfavourable prognostic factors, the use of steroid-sparing agents such as azathioprine, methotrexate, or rituximab is necessary, in order to minimise corticosteroid dosage and prevent side effects. Current studies indicate that combination therapy with prednisone and immunomodulators may be beneficial. Recent developments in treatment, such as the use of Inebilizumab, a monoclonal antibody, show promising results in reducing relapse rates.