Maintaining remission in systemic vasculitides

Immunoglobulin A vasculitis and Kawasaki disease are managed differently; see specific management in the separate topics.

Remission of disease activity in systemic vasculitides is defined as:

  • absence of clinical features of vasculitis
  • resolution of organ changes or stabilisation of organ structure and function
  • normalisation of inflammatory markers (serum C-reactive protein [CRP] concentration, erythrocyte sedimentation rate [ESR]).

A reasonable maintenance regimen is the lowest effective daily dose, started within 6 months of achieving remission. The optimal duration of therapy is unknown; however, to reduce the risk of disease relapse, maintenance oral corticosteroid therapy should be continued for at least 2 years once remission is achieved.

Tapering of long-term oral corticosteroids can be started when remission has been achieved, or if corticosteroid adverse effects develop. The rate of tapering depends on the clinical and inflammatory-marker response and any adverse effects. An example of tapering and stopping long-term, high-dose oral corticosteroids can be found in Example of tapering long-term, high-dose oral corticosteroids for systemic vasculitides.

Figure 1. Example of tapering long-term, high-dose oral corticosteroids for systemic vasculitides

Stone, 2017

Presentation:

A patient with a systemic vasculitis has been taking prednisolone 80 mg orally daily as induction therapy. Their symptoms have resolved, organ function has stabilised, and inflammatory markers (ESR and CRP) have normalised. Start a dose reduction until one of the following occurs:

  • the drug is stopped [NB1]
  • the patient remains asymptomatic on the lowest dose possible
  • the symptoms relapse and the dose must be increased again [NB2].

Suggested oral corticosteroid tapering regimen:

  • Induction dose 80 mg daily
  • Weeks 1 to 4—each week, reduce the daily dose by 10 mg (ie 70, 60, 50, 40 mg)
  • Weeks 5 to 9—each week, reduce the daily dose by 5 mg (ie 35, 30, 25, 20, 15 mg)
  • Week 10—each fortnight, reduce the daily dose by 2.5 mg (ie 12.5, 12.5 mg)
  • Week 12—for 1 week, reduce the daily dose by 2.5 mg (ie 10 mg)
  • Weeks 13 to 17—each week, reduce the daily dose by 1 mg (ie 9, 8, 7, 6, 5 mg)
  • Weeks 18 onwards—each fortnight, reduce the daily dose by 1 mg (ie 4, 4, 3, 3, 2, 2, 1, 1 mg).
Note:

CRP = serum C-reactive protein concentration; ESR = erythrocyte sedimentation rate

NB1: Patients with giant cell arteritis can usually taper off corticosteroids completely over 18 months to 2 years. Most patients with other systemic vasculitides usually cannot completely stop oral corticosteroids and need to stay on a low dose in combination with another immunomodulatory drug long term.

NB2: The optimal duration of therapy is not known and varies in individuals.

Long-term oral corticosteroid therapy is associated with significant adverse effects; for more information and advice on how to minimise and monitor for adverse effects, see Specific considerations for use of systemic corticosteroids.

Oral corticosteroids are usually used in combination with another immunomodulatory drug (eg azathioprine, cyclophosphamide, methotrexate, rituximab) for their corticosteroid-sparing effect and maintenance of remission. For more information about immunomodulatory drugs, see Principles of immunomodulatory drug use.

When remission of disease activity is achieved, monitor all patients with systemic vasculitis for evidence of disease recurrence. See the specific topics for advice on recommended follow-up.