How is IgG4 RD treated
Consistent with hypotheses implicating allergic and autoimmune elements to pathogenesis, many cases and manifestations are initially responsive to glucocorticoids, typically started at high doses and continued over months-long tapering regimens for organ-threatening, symptomatic disease.
Patients may relapse or tolerate steroids poorly requiring other immunosuppressive medications to be added for their steroid-sparing effect.
Rituximab is frequently used as the preferred steroid-sparing agent (supported by case-series data).
Surgical repair or endovascular aneurysm repair is required for large aneurysms which are at increased risk for rupture when treated with high-dose glucocorticoid therapy.
