Table 1

Recommendation statements

StatementLevel of evidenceGrade of recommendation
1. We recommend that patients with AAV are managed in close collaboration with, or at, centres of expertise.3C
2. A positive biopsy is strongly supportive of a diagnosis of vasculitis and we recommend biopsies to assist in establishing a new diagnosis and for further evaluation for patients suspected of having relapsing vasculitis.3C
3. For remission-induction of new-onset organ-threatening or life-threatening AAV we recommend treatment with a combination of glucocorticoids and either cyclophosphamide OR rituximab.1 for GPA/MPA, 3 for EGPAA for GPA/MPA, C for EGPA
4. For remission-induction of non-organ-threatening AAV we recommend treatment with a combination of glucocorticoids and either methotrexate or mycophenolate mofetil*.1BB for MTX, C for MMF
5. For a major relapse of organ-threatening or life-threatening disease in AAV we recommend treatment as per new disease with a combination of glucocorticoids and either cyclophosphamide OR rituximab.1 for GPA/MPA, 3 for EGPA and CYC, 4 for EGPA and RTXA for GPA/MPA, C for EGPA and CYC, C for EGPA and RTX
6. (i) Plasma exchange should be considered for patients with AAV and a serum creatine level of ≥500 µmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease.1BB
6. (ii) Plasma exchange can also be considered for the treatment of severe diffuse alveolar haemorrhage.3C
7. For remission-maintenance of AAV we recommend treatment with a combination of low-dose glucocorticoids and either azathioprine, rituximab, methotrexate or mycophenolate mofetil*.1B for GPA/MPA
3 for EGPA and AZA
A for GPA/MPA, C for EGPA and AZA
8. We recommend that remission-maintenance therapy for AAV be continued for at least 24 months following induction of sustained remission.4D
9. For patients with AAV refractory to remission-induction therapy we recommend switching from cyclophosphamide to rituximab or from rituximab to cyclophosphamide. These patients should be managed in close conjunction with, or referred to, an expert centre for further evaluation and potential enrolment in clinical trials.3C
10. We recommend that structured clinical assessment rather than ANCA testing should inform decisions on changes in treatment for AAV.4D
11. We recommend the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide.2BC
12. Hypoimmunoglobulinaemia has been noted after treatment with rituximab. We recommend testing of serum immunoglobulin levels prior to each course of rituximab and in patients with recurrent infection.3C
13. We recommend periodic assessment of cardiovascular risk for patients with AAV.2BB
14. We recommend that patients with AAV should be given a clear verbal explanation of the nature of their disease, the treatment options, the side effects of treatment, and the short-term and long-term prognoses.3C
15. We recommend that following the remission-induction phase of treatment, patients with AAV be assessed for the extent and ongoing impact of comorbidities associated with their diagnosis. Patients should then be advised where they might find the necessary therapies or support for these conditions.4D
  • *The drugs are listed in order of the strength of vote (see text).

  • AAV, ANCA-associated vasculities; ANCA, antineutrophil cytoplasmic antibody; AZA, azathioprine; CYC, cyclophosphamide; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MMF, mycophenolate mofetil; MPA, microscopic polyangiitis; MTX, methotrexate; RTX, rituximab.