Vasculitis Treatment Approaches

Classification and Treatment

The American College of Rheumatology recommends categorizing ANCA-associated vasculitis patients by severity for treatment decisions, including localized, early systemic, generalized, severe, and refractory disease 1, 2
The European League Against Rheumatism suggests that combination therapy with cyclophosphamide and glucocorticoids is the standard induction regimen for generalized and severe disease 1, 3, 2
The American College of Rheumatology recommends methotrexate plus glucocorticoids as a less toxic alternative for early systemic disease 1, 3

The American College of Rheumatology recommends cyclophosphamide 2 mg/kg/day orally and glucocorticoids 1 mg/kg/day for induction therapy in generalized and severe disease 1, 2
The European League Against Rheumatism suggests that azathioprine is the first-line maintenance agent after remission is achieved 3
The American College of Rheumatology recommends rituximab as an alternative first-line agent for patients concerned about fertility or with relapsing disease 2

The American College of Rheumatology recommends mandatory prophylaxis, including pneumocystis jiroveci prophylaxis with trimethoprim/sulfamethoxazole and mesna with cyclophosphamide to prevent hemorrhagic cystitis 1, 2
The European League Against Rheumatism suggests that monitoring parameters include complete blood count, renal function, blood glucose, and urinalysis 1, 2

The American Heart Association recommends that patients with primary CNS vasculitis receive corticosteroids and cytotoxic agents 5, 6
The American College of Rheumatology recommends that patients with Takayasu arteritis receive non-biological glucocorticoid-sparing agents in combination with glucocorticoids 2
The European League Against Rheumatism suggests that patients with Churg-Strauss syndrome be treated similarly to other ANCA-associated vasculitides 1, 3

The American College of Rheumatology recommends surveillance for cyclophosphamide-related bladder cancer risk and other long-term complications, including cardiovascular disease, neoplasia, cerebrovascular events, renal dysfunction, and metabolic abnormalities 3, 7