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Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis

Author
Ronald J Falk, MD
Section Editors
Gerald B Appel, MD
Fernando C Fervenza, MD, PhD
Deputy Editor
Albert Q Lam, MD

TERMINOLOGY

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].

INTRODUCTION

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

Therapy of GPA and MPA has two components: induction of remission with initial immunosuppressive therapy; and maintenance immunosuppressive therapy for a variable period to prevent relapse.

Maintenance immunosuppressive therapy of GPA and MPA will be reviewed here. Initial immunosuppressive therapy, the treatment of resistant or relapsing disease, clinical manifestations and diagnosis, and prognosis are discussed elsewhere. (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Treatment-resistant granulomatosis with polyangiitis and microscopic polyangiitis" and "Identification and management of relapsing disease in granulomatosis with polyangiitis and microscopic polyangiitis" and "Clinical manifestations and diagnosis of 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".)

GOAL OF MAINTENANCE THERAPY

After attainment of remission with initial immunosuppressive therapy, almost all patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) are switched to a maintenance regimen, most often azathioprine, rituximab, or methotrexate. Some may not require maintenance therapy, including those with drug-induced vasculitis and some who are MPO-antineutrophil cytoplasmic autoantibody (ANCA) positive and have a clinical remission after induction therapy. (See 'Patients with drug-induced ANCA' below and 'MPO-ANCA-positive patients' below.)

                         

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Literature review current through: Nov 2016. | This topic last updated: Wed Jan 13 00:00:00 GMT+00:00 2016.
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References
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