Identification and management of relapsing disease 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
- Section Editors
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Gerald B Appel, MD
Gerald B Appel, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Columbia University College of Physicians and Surgeons
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), and both have similar features on renal histology (eg, a focal necrotizing, pauci-immune glomerulonephritis). However, there are several differences between these disorders. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)
Patients who have vasculitis associated with ANCA (ie, GPA and MPA) can also be distinguished according to the two most prominent ANCA serotypes, specifically, proteinase-3 (PR3) and myeloperoxidase (MPO). The ANCA serotype may have more prognostic significance than the terms "MPA" and "GPA" with respect to response to therapy, propensity for relapse, and patient outcome. In addition, genetic variants that are associated with ANCA disease are more specifically linked with the antigen (PR3 versus MPO) rather than with the clinical phenotype. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis", section on 'Limitations of classification algorithms'.)
Between 70 and 90 percent of patients with GPA or MPA attain clinical remission with initial immunosuppressive therapy (typically, cyclophosphamide or rituximab in combination with glucocorticoids). They are then treated with maintenance immunosuppressive therapy, usually with azathioprine, rituximab, or methotrexate, for a period that is typically 12 to 18 months. However, relapses are common, and some patients have frequent relapses. In addition, among patients who progress to end-stage renal disease, nonrenal relapses can occur in patients treated with maintenance hemodialysis or renal transplantation, and renal manifestations can recur in the transplanted kidney.
Issues related to relapsing disease in patients with GPA or MPA will be reviewed here. This includes patients who have progressed to end-stage renal disease and are on chronic renal replacement therapy.
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- NEW TERMINOLOGY
- DEFINITION OF RELAPSING DISEASE
- IDENTIFYING PATIENTS WITH RELAPSING DISEASE
- Monitoring for relapse
- - Patient self-monitoring
- - Monitoring by the clinician
- ANCA titers
- Risk factors for relapse
- Precipitants of relapse
- Diagnosis of relapsing disease
- - Differential diagnosis
- TREATMENT OF RELAPSING DISEASE
- Overall approach
- Treatment of a severe relapse
- Treatment of a mild relapse
- - Disease limited to the upper respiratory tract
- Patients who have multiple relapses
- Maintenance therapy after reinduction for a severe relapse
- EFFECT OF RELAPSE ON PROGNOSIS
- SUMMARY AND RECOMMENDATIONS