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Alternative agents in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

Ronald J Falk, MD
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editor
Albert Q Lam, MD


In January 2011, the Boards of Directors of the American College of Rheumatology (ACR), the American Society of Nephrology (ASN), and the European League Against Rheumatism (EULAR) recommended that the name "Wegener's granulomatosis" be changed to "granulomatosis with polyangiitis," abbreviated as GPA [1-3]. This change reflects a plan to gradually shift from honorific eponyms to a disease-descriptive or etiology-based nomenclature.


Granulomatosis with polyangiitis, which can be abbreviated as GPA, and microscopic polyangiitis (MPA) are related systemic vasculitides. Both are associated with antineutrophil cytoplasmic antibodies (ANCA), have similar features on renal histology (eg, a focal necrotizing, pauci-immune glomerulonephritis), and have similar outcomes. There are, however, several differences between these disorders. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis", section on 'Clinical presentation'.)

Patients with GPA or MPA are initially treated with immunosuppressive therapy to induce remission, most often consisting of glucocorticoids in combination with either cyclophosphamide or rituximab. Patients who attain remission are then treated with azathioprine or methotrexate, which are less toxic than cyclophosphamide, and low-dose glucocorticoids to maintain the remission. The usual duration of maintenance therapy after stable remission has been induced is 12 to 18 months. More prolonged maintenance therapy should be considered in patients who have multiple disease relapses. (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis".)

Relapses occur either on maintenance therapy or after maintenance therapy has been discontinued. Patients who have more than minor relapses while on maintenance therapy and relapses occurring in patients who are no longer on maintenance immunosuppression are usually treated with reinstitution of the initial induction regimen. (See "Identification and management of relapsing disease in granulomatosis with polyangiitis and microscopic polyangiitis".)

Other therapies that have been evaluated for the induction and maintenance of remission and the treatment of relapses in patients with GPA or MPA will be reviewed here. The clinical manifestations and diagnosis of these disorders, the treatment of cyclophosphamide-resistant disease, and patient and renal outcomes are discussed elsewhere. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Treatment-resistant granulomatosis with polyangiitis and microscopic polyangiitis" and "Prognosis in granulomatosis with polyangiitis and microscopic polyangiitis, and management of those who develop end-stage renal disease".)

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Literature review current through: Nov 2017. | This topic last updated: Mar 03, 2017.
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