2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (Part 10)

Allow me to continue with our discussion of the guidelines of the American College of Rheumatology (ACR) in relation to the antineutrophil cytoplasmicantibody (ANCA)–associated vasculitides (AAV).

We mentioned previously that ANCA-AAV is a group of diseases composed of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

For patients with severe GPA/MPA and the disease has entered remission using cyclophosphamide or rituximab, it is recommended to use methotrexate or azathioprine for remission maintenance.

Methotrexate or azathioprine treatment is better than leflunomide based on the data that methotrexate and azathioprine are best for maintenance of remission.

The data for leflunomide are more limited. In a trial comparing leflunomide to methotrexate, it is shown that using leflunomide demonstrated a decreased rate of relapse but a higher rate of drug withdrawal.

The trial used a dose of 30 mg/day, which may have contributed to toxicity. Next week we will be having a new topic in this column.