ANCA vasculitis maintenance — SCE Rheumatology MCQ

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HardVasculitisANCA vasculitis maintenanceSCE Rheumatology

A 57-year-old man with PR3-ANCA-positive granulomatosis with polyangiitis had renal and pulmonary involvement at diagnosis. He subsequently relapsed while receiving azathioprine maintenance and has now achieved a second complete remission after rituximab re-induction. His eGFR is stable, serum IgG is normal, and hepatitis B screening shows no evidence of current or previous infection. Which biologic is most appropriate for remission maintenance?

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Correct answer: B — Rituximab

Rituximab is the preferred biologic for remission maintenance in granulomatosis with polyangiitis. This patient has a particularly relapse-prone phenotype: PR3-ANCA positivity, previous major-organ disease and relapse during azathioprine treatment. RITAZAREM demonstrated that repeat-dose rituximab after rituximab re-induction was superior to azathioprine in relapsing ANCA-associated vasculitis. Normal IgG and negative hepatitis B screening provide reassurance against important safety barriers, although immunoglobulins and infections require ongoing monitoring. Mepolizumab has a maintenance role in relapsing or refractory eosinophilic granulomatosis with polyangiitis, not conventional PR3-positive GPA. Etanercept has no established maintenance role in GPA, while ustekinumab and belimumab are not recommended treatments for ANCA-associated vasculitis.

Reference: Hellmich B et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Recommendation on remission maintenance in GPA/MPA. Ann Rheum Dis. 2024;83:30–47. https://pubmed.ncbi.nlm.nih.gov/36927642/