Questions informing the literature review for updated recommendations.
| Question | Vote, n = 19 |
|---|---|
| Which of indirect immunofluorescence and/or high-quality immunoassay should be recommended for ANCA detection in patients with suspected AAV? | 100% |
| In patients undergoing induction therapy for severe AAV, does pulse methylprednisolone confer clinical benefit compared to no pulse? | 100% |
| In patients undergoing induction therapy for GPA or MPA, can a “reduced dose” GC tapering protocol be used instead of a “standard” GC tapering protocol? | 100% |
| In patients undergoing induction therapy for severe GPA and MPA, does plasma exchange confer clinical benefit compared to no plasma exchange? | 100% |
| In patients undergoing induction therapy for GPA and MPA, does MMF have comparable clinical efficacy to CYC? | 100% |
| In patients with severe AAV, which maintenance agents are effective? | 100% |
| In patients with GPA and MPA, does extended maintenance therapy (48 months) lead to fewer relapses compared to standard (24 months) maintenance therapy? | 100% |
| In patients with GPA and MPA, which RTX maintenance regimens are effective? | 100% |
| In patients with GPA and MPA receiving RTX, should gamma globulins be monitored and how should hypogammaglobulinemia be managed? | 100% |
| In patients with GPA and MPA receiving RTX, should B cells (CD19) be monitored to determine the risk of relapse? | 95% |
| In patients with AAV, should ANCA be monitored to determine the risk of relapse? | 100% |
| In patients with nonsevere EGPA or MPA (FFS = 0), does the addition of conventional immunosuppressants to GC improve remission or relapse rate? | 95% |
| In patients with EGPA, is there a role for biological therapies for induction or maintenance? | 95% |
| In patients receiving RTX or CYC for AAV, does TMP-SMX prophylaxis reduce severe infection risk? | 95% |
| In patients with AAV, which vaccinations are safe, effective, and recommended? | 95% |
| Additional topics of interest included in the literature search, suggested by ≥ 2 CanVasc members | |
| In patients with GPA or MPA, does the combination of RTX + CYC for induction confer clinical benefit? | |
| In patients with AAV, what is the optimal duration of low-dose GC during maintenance? | |
| In patients with AAV, is there a maximum recommended lifetime cumulative dose of CYC? | |
| In patients with GPA or MPA with subglottic stenosis, which medical treatments are effective? | |
| In patients with AAV, what is the efficacy of other biologic therapies or RTX biosimilars? |
AAV: ANCA-associated vasculitis; ANCA: antineutrophil cytoplasm antibody; AZA: azathioprine; CD19: cluster of differentiation 19; CYC: cyclophosphamide; EGPA: eosinophilic granulomatosis with polyangiitis; FFS: Five Factor Score; GC: glucocorticoid; GPA: granulomatosis with polyangiitis; MMF: mycophenolate mofetil; MPA: microscopic polyangiitis; RTX: rituximab; TMP-SMX: trimethoprim-sulfamethoxazole.