The CARI guidelines. Induction and maintenance therapy in ANCA-associated systemic vasculitis.

Induction therapy a. Combination treatment with cyclophosphamide plus prednisolone should be used for induction of disease remission when organ function is threatened. Both intravenous (IV) and oral cyclophosphamide show equal clinical efficacy and toxicity. (Level II evidence) b. Methotrexate is an alternative to cyclophosphamide-based induction therapy in patients with milder, early disease (serum creatinine <150 mmol/L). (Level II evidence) c. Plasma exchange should be preferred to pulse methylprednisolone as an adjunctive therapy in the initial treatment of severe ANCA-associated systemic vasculitis (AASV) causing necrotising glomerulonephritis and acute kidney failure (creatinine >500 mmol/L). (Level II evidence)