Treatment guidelines

A framework of practice for the treatment and management of AAV patients.1

Treatment pathway


AAV is a long-term condition with a relapsing
course that needs long-term immunosuppresive therapy2-10

  • Organ- or life-threatening disease
  • Remission
  • Relapse
  • Refractory
Vasculitis treatment pathway of organs or life threatening disease chart Vasculitis treatment pathway of organs or life threatening disease mobile chart

The balance of treatment


Treatment must balance controlling vasculitis with minimising treatment-related damage1–4

At the time of AAV diagnosis or relapse, the major concerns around treatment involve controlling vasculitis activity and minimising the acute adverse effects from therapy.1,2 Later when remission is achieved, cumulative organ damage, often related to long-term low dose glucocorticoids and patient experience are more important.1–4

EULAR/ERA-EDTA recommend different treatment combinations for two grades of disease severity (organ- or life-threatening AAV and non-organ-threatening AAV) seen in AAV.1 Real-world data confirm that initial remission induction therapy varies according to disease severity, although glucocorticoids are used frequently across all severities:5*†

Organ- or life-threatening AAV
- Severe: 72.4% cyclophosphamide, 28.4% rituximab and 86.8% glucocorticoids
- Moderate: 49.4% cyclophosphamide, 29.6% rituximab and 80.4% glucocorticoids

Non-organ-threatening AAV
- Mild: 23.2% cyclophosphamide, 26.1 % rituximab, 80.4% glucocorticoids, 13.8% methotrexate, 8% mycophenolate mofetil and 8% azathioprine

Physicians managing AAV will need to determine their treatment approach based on an assessment of disease severity, and balance achieving remission quickly but also avoiding the adverse effects from therapy.1–5

The relapsing patient is a particular challenge as they present with acute vasculitis on the back of chronic organ damage from vasculitis and long term glucocorticoids – treatment decisions can be very difficult.1,2

Different physicians will manage patients with different underlying type (MPA vs GPA), severity and frequency of renal involvement. As such, rheumatologists, nephrologists and other specialists will experience different clinical challenges.6

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Clinical service case study6‡

A retrospective study of patients presenting with AAV to the rheumatology (n=15) or nephrology (n=30) department of the same hospital, from June 2002 to July 2011.
The nephrology patient cohort was older, and had a higher mean BVAS score and median ANCA titer.

The rheumatology cohort more commonly presented with lung, neurological, ENT and ocular symptoms.

Induction treatment varied between cohorts, with the most frequent being:
– Rheumatology patients: oral corticosteroids (73%) and methotrexate (60%)
– Nephrology patients: intravenous and oral corticosteroids (93%) and cyclophosphamide (90%)

• Methotrexate is contraindicated in patients with significant renal impairment, which was reflected in the choice of induction treatment used by the two study groups6,7

• 53% of rheumatology patients vs 30% of nephrology patients who completed induction therapy relapsed

• Nephrology patients had a higher complication rate, including infection, malignancy and mortality

• AAV is a heterogenous disease that is managed differently by the two different disciplines. Interdisciplinary communication and cooperation are essential for successful management

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References & footnotes



Real-world treatment data demonstrates that AEs remain an issue for new and relapsing patients1*


Over the first 12 months of treatment in patients with GPA or MPA, AEs are common. At this time, glucocorticoid dose is typically highest, as current treatment guidelines recommend the use of GCs for remission-induction.1,2

New-patients (n=929)1

Graph showing frequency of Adverse events in new patients month by month. Graph showing frequency of Adverse events in new patients month by month. Graph showing frequency of Adverse events in new patients month by month. Graph showing frequency of Adverse events in new patients month by month.

Relapsing-patients (n=268)1

Graph showing frequency of Adverse events in relapsing patients month by month. Graph showing frequency of Adverse events in relapsing patients month by month. Graph showing frequency of Adverse events in relapsing patients month by month. Graph showing frequency of Adverse events in relapsing patients month by month.