Table 1.
Recommendation statements
| Statement | Level of evidence | Grade of recommendation | |||||
| Task force (n=21) | EUVAS (n=88) | Combined (n=109) | |||||
| Mode | Median | Mode | Median | Mode | Median | ||
|---|---|---|---|---|---|---|---|
| 1. We recommend that patients with ANCA-associated vasculitis are managed in close collaboration with, or at, centres of expertise. | 3 | C | C | C | C | C | C |
| 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. | 3 | C | C | C | C | C | C |
| 3. For remission induction of new-onset organ or life-threatening ANCA-associated vasculitis we recommend treatment with a combination of glucocorticoids and either cyclophosphamide or rituximab. | |||||||
| cyclophosphamide and GPA/MPA | 1 | A | A | A | A | A | A |
| cyclophosphamide and EGPA | 3 | C | C | C | C | C | C |
| rituximab and GPA/MPA | 1 | A | A | A | A | A | A |
| rituximab and EGPA | 3 | C | C | D | D | D | D |
| 4. For remission induction of non-organ-threatening ANCA-associated vasculitis we recommend treatment with a combination of glucocorticoid and either methotrexate or mycophenolate mofetil*. | |||||||
| methotrexate | IB | B | B | B | B | B | B |
| mycophenolate mofetil | IB | C | C | C | C | C | C |
| 5. For a major relapse of organ-threatening or life-threatening disease in ANCA-associated vasculitis we recommend treatment as per new disease with a combination of glucocorticoids and either cyclophosphamide or rituximab. | |||||||
| cyclophosphamide and GPA/MPA | 1 | A | A | A | A | A | A |
| cyclophosphamide and EGPA | 3 | C | C | C | C | C | C |
| rituximab and GPA/MPA | 1 | A | A | A | A | A | A |
| 6.i. Plasma exchange should be considered for patients with ANCA-associated vasculitis and a serum creatinine level of greater than 500 µmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease. | 1B | B | B | B | B | B | B |
| 6.ii. Plasma exchange can also be considered for the treatment of severe diffuse alveolar haemorrhage. | 3 | C | C | C | C | C | C |
| 7. For remission maintenance of ANCA-associated vasculitis we recommend treatment with a combination of low-dose glucocorticoids and either azathioprine,rituximab,methotrexate or mycophenolate mofetil*. | |||||||
| azathioprine and GPA/MPA | 1B | A | A | A | A | A | A |
| azathioprine and EGPA | 3 | C | C | C | C | C | C |
| rituximab and GPA/MPA | 1B | A | A | A | A | A | A |
| methotrexate and GPA/MPA | 1B | A | A | B | B | B | B |
| mycophenolate mofetil and GPA/MPA | 1B | A | A | B | B | B | B |
| 8. We recommend that remission-maintenance therapy for ANCA-associated vasculitis be continued for at least 24 months following induction of sustained remission. | 4 | D | D | NA | NA | NA | NA |
| 9. For patients with ANCA-associated vasculitis 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. | 3 | C | C | C | C | C | C |
| 10. We recommend that structured clinical assessment rather than ANCA testing should inform decisions on changes in treatment for ANCA-associated vasculitis. | 4 | D | D | NA | NA | NA | NA |
| 11. We recommend the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide. | 2B | C | C | C | C | C | C |
| 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. | 3 | C | C | C | C | C | C |
| 13. We recommend periodic assessment of cardiovascular risk for patients with ANCA-associated vasculitis. | 2B | B | B | B | B | B | B |
| 14. We recommend that patients with ANCA-associated vasculitis 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 prognosis. | 3 | C | C | C | C | C | C |
| 15. We recommend that following the remission-induction phase of treatment, patients with ANCA-associated vasculitis 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. | 4 | D | D | NA | NA | NA | NA |
*The drugs are listed in order of the strength of vote.
ANCA, antineutrophilic cytoplasmic antibodies; EGPA, eosinophilic granulomatosis with polyangiitis; EUVAS, European Vasculitis Society; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; NA, not applicable.