What is the role of induction therapy in the treatment of ANCA-associated vasculitis?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Optimal induction therapy for patients with generalized disease (renal or other major organ involvement) is a subject of intensive study. Initial guidelines suggested a combination of cyclophosphamide and high-dose glucocorticoids. However, there have been 3 randomized controlled trials investigating the use of rituximab as an induction agent in adults with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). [38, 39, 40] These studies have shown that rituximab is likely as effective as cyclophosphamide in inducing remission.

The most recent treatment recommendations [37] suggest that either regimen be considered and that rituximab may be preferred when cyclophosphamide avoidance is desired (eg, due to toxicity). Some evidence suggests that granulomatous manifestations (eg, orbital granulomas) may not respond to rituximab as well as vasculitic manifestations. [41] With either regimen, high-dose prednisone (1 mg/kg) should be maintained for 1 month. When rapid effect is needed, intravenous (IV) pulsed methylprednisolone may be used in addition to the oral prednisone.

A study by van Daalen et al reported that rituximab treatment for ANCA-associated vasculitis had lower malignancy risk than in cyclophosphamide treatment and that rituximab was not associated with an increased malignancy risk compared with the general population. [42]

Local guidelines for the prevention of glucocorticoid-induced osteoporosis should be followed in all patients. Cyclophosphamide use should be limited to 3-6 months because of potential for long-term toxicity. However, no consensus about whether pulse IV cyclophosphamide is superior to daily oral therapy. All patients who receive cyclophosphamide should also receive prophylaxis against Pneumocystis jiroveci (trimethoprim-sulfamethoxazole or pentamidine), especially those with GPA.

For patients with mild-to-moderate or limited disease, methotrexate can be used as a less toxic alternative for induction. However, there is some evidence that induction with methotrexate may be associated with a higher risk of relapse. [43]

Plasma exchange is recommended as adjunctive therapy for patients with rapidly progressive severe renal disease.

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