Table 8 The 15 recommendations for the management of small and medium vessel vasculitis with the level of evidence for each statement and the median strength of recommendation as per European League Against Rheumatism (EULAR) operating procedures
StatementLevel of evidenceMedian vote
1. We recommend that patients with primary small and medium vessel vasculitis be managed in collaboration with, or at centres of expertise3D
2. We recommend that ANCA testing (including indirect immunofluorescence and ELISA) should be performed in the appropriate clinical context1AA
3. A positive biopsy is strongly supportive of vasculitis and we recommend the procedure to assist diagnosis and further evaluation for patients suspected of having vasculitis3C
4. We recommend the use of a structured clinical assessment, urine analysis and other basic laboratory tests at each clinical visit for patients with vasculitis3C
5. We recommend that patients with ANCA-associated vasculitis be categorised according to different levels of severity to assist treatment decisions2BB
6. We recommend a combination of cyclophosphamide (intravenous or oral) and glucocorticoids for remission induction of generalised primary small and medium vessel vasculitis.1A for WG and MPAA for WG and MPA
1B for PAN and CSSA for PAN and CSS
7. We recommend a combination of methotrexate (oral or parenteral) and glucocorticoid as a less toxic alternative to cyclophosphamide for the induction of remission in non-organ threatening or non-life threatening ANCA-associated vasculitis1BB
8. We recommend the use of high-dose glucocorticoids as an important part of remission induction therapy3C
9. We recommend plasma exchange for selected patients with rapidly progressive severe renal disease in order to improve renal survival1BA
10. We recommend remission-maintenance therapy with a combination of low-dose glucocorticoid therapy and, either azathioprine, leflunomide or methotrexate1B for azathioprineA for azathioprine
1B for leflunomideB for leflunomide
2B for methotrexateB for methotrexate
11. Alternative immunomodulatory therapy choices should be considered for patients who do not achieve remission or relapse on maximal doses of standard therapy: these patients should be referred to an expert centre for further management and enrolment in clinical trials3C
12. We recommend immunosuppressive therapy for patients with mixed essential cryoglobulinemic vasculitis (non-viral)4D
13. We recommend the use of antiviral therapy for the treatment of hepatitis C-associated cryoglobulinaemic vasculitis1BB
14. We recommend a combination of antiviral therapy, plasma exchange and glucocorticoids for hepatitis B-associated PAN3C
15. We recommend the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide2BC
  • ANCA, anti-neutrophilic cytoplasmic antibodies; CSS, Churg–Strauss syndrome; MPA, microscopic polyangiitis; PAN, polyarteritis nodosa; WG, Wegener granulomatosis.