Intensive induction therapy for severe systemic vasculitides

Intensive induction therapy is indicated for severe features of systemic vasculitides. The regimens used vary between the specific vasculitides, depending on the organ(s) affected and the extent of damage. The regimens typically include high-dose systemic corticosteroids and immunomodulatory drugs.

High-dose systemic corticosteroids are the mainstay of intensive induction therapy for severe systemic vasculitides. They are often used for the duration of treatment and sometimes in combination with other immunomodulatory drugs. Typical regimens may include:

  • high-dose systemic corticosteroids (eg pulse intravenous methylprednisolone, oral prednisolone or prednisone)
  • high-dose systemic corticosteroids plus cyclophosphamide
  • high-dose systemic corticosteroids plus a biological disease-modifying antirheumatic drug (bDMARD) such as rituximab or tocilizumab.

High-dose corticosteroid induction therapy typically comprises pulse intravenous methylprednisolone followed by high-dose oral corticosteroids. When intravenous methylprednisolone cannot be started immediately, high-dose oral corticosteroids can be started first and continued until intravenous methylprednisolone is available.

Cyclophosphamide is a conventional-synthetic disease-modifying antirheumatic drug (csDMARD) that may be used for intensive induction therapy for systemic vasculitis, typically in combination with systemic corticosteroids. Consider other drugs for people of childbearing potential because of cyclophosphamide’s effects on fertility.

Other csDMARDs that may be used include methotrexate and mycophenolate.

Rituximab is a B cell–targeting biological (b)DMARD that is sometimes used first line for intensive induction therapy in patients with microscopic polyangiitis or granulomatosis with polyangiitisYates, 2016. It is typically used in combination with systemic corticosteroids. Rituximab is not inferior to cyclophosphamide and is generally chosen for patients who:

  • have not responded to cyclophosphamide
  • have had, or are likely to have, major toxicity from cyclophosphamide
  • wish to conceive in the future and are concerned about the effect of cyclophosphamide on fertility.

Tocilizumab is an interleukin-6–targeting bDMARD that may also be used for intensive induction therapy in giant cell arteritis (GCA) in combination with systemic corticosteroids, instead of cyclophosphamideHellmich, 2020.