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British rheumatology experts have proposed guidelines for managing adults with ANCA-associated vasculitides (AAVs) -- granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis.
Ntatsaki E, Carruthers D, Chakravarty K, et al., on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis.Rheumatology (2014) doi:10.1093/rheumatology/ket445 [first published online April 11, 2014]
British rheumatology experts have proposed guidelines for managing adults with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAVs) -- granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis.
While definitions of the three AAVs were updated in 2012, there are still no validated diagnostic criteria. However, the working group of the British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) says all AAV patients should be considered to have severe, potentially life- or organ-threatening disease and be diagnosed and treated as quickly as possible.
Six disease-state categories were proposed for monitoring patients:
• Remission (well-controlled disease): “on-drug” remission with prednisolone of 10 mg/day or less and a Birmingham Vasculitis Activity Score (BVAS) of 1 or lower for 6 months.
• Drug-free remission is 6 months off of all treatment.
• Relapsing: previously well-controlled disease (with or without drugs) that has become active.
• Minor relapse: an increase of one or more new or worse minor items and no major BVAS items.
• Major relapse: an increase of one or more major BVAS item.
• Refractory disease: progressive disease unresponsive to current therapy.
Key points of the therapy recommendations:
The working group recommends that all newly diagnosed patients be given induction therapy either with glucocorticoids (GCs) and rituximab, or intravenous pulses of cyclophosphamide (CYC) with GCs (usually daily oral prednisolone).
• GC infusions of methylprednisolone are sometimes given just prior to or with the first two CYC pulses.
• Methotrexate (MTX) and mycophenolate mofetil (MMF) can be used as alternatives if there is low disease activity or no evident organ damage.
If the patient achieves remission, CYC should be stopped and azathioprine (AZA), MTX, or RTX should be substituted for maintenance:
• MMF (CellCept) or leflunomide (Arava) may be given as maintenance therapy if a patient is intolerant to AZA or MTX.
• Maintenance should continue for at least 24 months.
• Patients with GPA or those who remain PR3-ANCA positive should continue on immunosuppressants for up to 5 years.
• If remission lasts for at least one year, a GC taper should be considered.
• After GC withdrawal, other maintenance drugs can be stopped after 6 months.
• Patients who relapse may need a second course of induction therapy.
• RTX is considered more effective than CYC for refractory diseease.
All patients should be followed to assess disease activity with validated tools such as the BVAS, Vasculitis Damage Index (VDI) and 36-item Short Form (SF-36) to assess disease activity, an tested by ELISA for ANCA every 6 months.
Full text of the BSR/BHPR guidelines is available at http://rheumatology.oxfordjournals.org/content/suppl/2014/04/11/ket445.DC1/ket445-Full_Guidelines.pdf