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".)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:
- Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Arthritis Rheum 2011; 63:863.
- Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. J Am Soc Nephrol 2011; 22:587.
- Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis 2011; 70:704.
- Hogan J, Avasare R, Radhakrishnan J. Is newer safer? Adverse events associated with first-line therapies for ANCA-associated vasculitis and lupus nephritis. Clin J Am Soc Nephrol 2014; 9:1657.
- Robson J, Doll H, Suppiah R, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis 2015; 74:177.
- Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116:488.
- Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 2000; 43:1021.
- Matteson EL, Gold KN, Bloch DA, Hunder GG. Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort. Am J Med 1996; 101:129.
- Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001; 44:666.
- Slot MC, Tervaert JW, Franssen CF, Stegeman CA. Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement. Kidney Int 2003; 63:670.
- Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 2003; 41:776.
- Lionaki S, Hogan SL, Jennette CE, et al. The clinical course of ANCA small-vessel vasculitis on chronic dialysis. Kidney Int 2009; 76:644.
- Wallace ZS, Lu N, Miloslavsky E, et al. Nationwide Trends in Hospitalizations and In-Hospital Mortality in Granulomatosis With Polyangiitis (Wegener's). Arthritis Care Res (Hoboken) 2017; 69:915.
- Lai QY, Ma TT, Li ZY, et al. Predictors for mortality in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis: a study of 398 Chinese patients. J Rheumatol 2014; 41:1849.
- de Joode AA, Sanders JS, Stegeman CA. Renal survival in proteinase 3 and myeloperoxidase ANCA-associated systemic vasculitis. Clin J Am Soc Nephrol 2013; 8:1709.
- Hogan SL, Falk RJ, Chin H, et al. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med 2005; 143:621.
- Knight A, Askling J, Ekbom A. Cancer incidence in a population-based cohort of patients with Wegener's granulomatosis. Int J Cancer 2002; 100:82.
- Westman KW, Bygren PG, Olsson H, et al. Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 1998; 9:842.
- Faurschou M, Sorensen IJ, Mellemkjaer L, et al. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol 2008; 35:100.
- Weidner S, Geuss S, Hafezi-Rachti S, et al. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant 2004; 19:1403.
- Aasarød K, Iversen BM, Hammerstrøm J, et al. Wegener's granulomatosis: clinical course in 108 patients with renal involvement. Nephrol Dial Transplant 2000; 15:611.
- Aasarød K, Bostad L, Hammerstrøm J, et al. Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis. Nephrol Dial Transplant 2001; 16:953.
- Mekhail TM, Hoffman GS. Longterm outcome of Wegener's granulomatosis in patients with renal disease requiring dialysis. J Rheumatol 2000; 27:1237.
- Glassock RJ. Intensive plasma exchange in crescentic glomerulonephritis: help or no help? Am J Kidney Dis 1992; 20:270.
- Cole E, Cattran D, Magil A, et al. A prospective randomized trial of plasma exchange as additive therapy in idiopathic crescentic glomerulonephritis. The Canadian Apheresis Study Group. Am J Kidney Dis 1992; 20:261.
- Nachman PH, Hogan SL, Jennette JC, Falk RJ. Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996; 7:33.
- Geffriaud-Ricouard C, Noël LH, Chauveau D, et al. Clinical spectrum associated with ANCA of defined antigen specificities in 98 selected patients. Clin Nephrol 1993; 39:125.
- Hauer HA, Bajema IM, Van Houwelingen HC, et al. Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 2002; 62:1732.
- Neumann I, Kain R, Regele H, et al. Histological and clinical predictors of early and late renal outcome in ANCA-associated vasculitis. Nephrol Dial Transplant 2005; 20:96.
- Berden AE, Jones RB, Erasmus DD, et al. Tubular lesions predict renal outcome in antineutrophil cytoplasmic antibody-associated glomerulonephritis after rituximab therapy. J Am Soc Nephrol 2012; 23:313.
- Bomback AS, Appel GB, Radhakrishnan J, et al. ANCA-associated glomerulonephritis in the very elderly. Kidney Int 2011; 79:757.
- Lee T, Gasim A, Derebail VK, et al. Predictors of treatment outcomes in ANCA-associated vasculitis with severe kidney failure. Clin J Am Soc Nephrol 2014; 9:905.
- Nachman PH, Segelmark M, Westman K, et al. Recurrent ANCA-associated small vessel vasculitis after transplantation: A pooled analysis. Kidney Int 1999; 56:1544.
- Beck L, Bomback AS, Choi MJ, et al. KDOQI US commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis. Am J Kidney Dis 2013; 62:403.
- Knoll G, Cockfield S, Blydt-Hansen T, et al. Canadian Society of Transplantation consensus guidelines on eligibility for kidney transplantation. CMAJ 2005; 173:1181.
- Moran S, Little MA. Renal transplantation in antineutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Rheumatol 2014; 26:37.
- NEW TERMINOLOGY
- END-STAGE RENAL DISEASE (ESRD)
- Outcomes in elderly patients
- MANAGEMENT OF PATIENTS WHO DEVELOP ESRD
- Patients on maintenance dialysis
- - Importance of infection
- Renal transplantation
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- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS