Management for immunoglobulin G4 (IgG4)-related disease
Management for immunoglobulin G4 (IgG4)–related disease usually requires a collaborative approach with multiple specialists, depending on the presenting features of the disease.
In the initial inflammatory phase of IgG4–related disease, high-dose systemic corticosteroids are often effective and are used first line. If high-dose pulse intravenous corticosteroids are indicated for urgent treatment of organ- or life-threatening features of IgG4–related disease, use:
methylprednisolone sodium succinate 0.5 to 1 g intravenously, over 1 hour, daily for 3 days, then switch to oral prednisolone (or prednisone) as below. methylprednisolone sodium succinate methylprednisolone methylprednisolone
If high-dose oral corticosteroids are indicated for the management of IgG4–related disease, useLanzillotta, 2020Löhr, 2020:
prednisolone (or prednisone) 0.6 to 1 mg/kg up to 80 mg orally, daily in 2 divided doses. Continue until symptoms subside and inflammatory markers normalise, then taper slowly to maintain remission. prednis ol one prednis(ol)one prednis(ol)one
If people require long-term, high-dose corticosteroid therapy to prevent relapse, other immunomodulatory drugs may be considered for their corticosteroid-sparing effect. Immunomodulatory drugs used for IgG4–related disease may include conventional synthetic DMARDs such as azathioprine, mycophenolate, methotrexate, leflunomide, tacrolimus or ciclosporin, and rituximab.
Detailed international guidelines1 on the management of IgG4–related disease are available, but are largely based on case reports and retrospective studiesLanzillotta, 2020.