Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis
- Ronald J Falk, MD
Ronald J Falk, MD
- Nan and Hugh Cullman Eminent Professor of Nephrology
- Chair, Department of Medicine
- Director, UNC Kidney Center
- University of North Carolina-Chapel Hill
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Section Editors
- Gerald B Appel, MD
Gerald B Appel, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Columbia University College of Physicians and Surgeons
- Fernando C Fervenza, MD, PhD
Fernando C Fervenza, MD, PhD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Mayo Clinic College of Medicine
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].
Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are related systemic vasculitides. Both are associated with antineutrophil cytoplasmic autoantibodies (ANCA), have similar clinical manifestations and 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 GPA or 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 myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibody (ANCA) positive and have a clinical remission after induction therapy for their initial presentation with vasculitis. (See 'Patients with drug-induced ANCA-associated vasculitis' below and 'MPO-ANCA-positive patients' below.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- GOAL OF MAINTENANCE THERAPY
- WHEN TO START MAINTENANCE THERAPY
- If cyclophosphamide is used for induction
- If rituximab is used for induction
- SELECTING A REGIMEN FOR MAINTENANCE THERAPY
- General principles
- - Choosing a maintenance drug
- - Tapering of glucocorticoids started during induction
- - Prophylaxis against infection
- - Patients who require dialysis
- - Patients with drug-induced ANCA-associated vasculitis
- Newly diagnosed patients
- - PR3-ANCA-positive patients
- - MPO-ANCA-positive patients
- Patients who have had one or more relapses
- - Relapse occurred during maintenance therapy
- - Relapse occurred after completion of maintenance therapy
- DOSING OF MAINTENANCE THERAPY
- Azathioprine dosing
- Rituximab dosing
- Methotrexate dosing
- Mycophenolate mofetil dosing
- WHEN TO STOP MAINTENANCE THERAPY
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS