Prognosis in granulomatosis with polyangiitis and microscopic polyangiitis, and management of those who develop end-stage renal disease
- 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
- 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]. This change reflects a plan to gradually shift from honorific eponyms to a disease-descriptive or etiology-based nomenclature.
Granulomatosis with polyangiitis (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 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 and MPA are treated with initial immunosuppressive therapy (usually cyclophosphamide or rituximab, most commonly in association with glucocorticoids) to induce remission. This is followed by maintenance immunosuppressive therapy with azathioprine, methotrexate, or rituximab to sustain the remission. Although most patients respond to these measures, relapses can occur.
Despite the efficacy of immunosuppressive therapy, some patients have appreciable morbidity, and some patients die from the disease and/or adverse effects of drug therapy. In addition, some patients develop end-stage renal disease (ESRD) and require renal replacement therapy with dialysis or renal transplantation.
The prognosis in patients with GPA and MPA, as well as the management of patients who develop ESRD, will be reviewed in this topic. The clinical manifestations and diagnosis of these disorders, initial and maintenance immunosuppressive therapy, and the management of cyclophosphamide-resistant or relapsing disease are discussed separately. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Maintenance 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".)
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