Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis
- Andre A Kaplan, MD
Andre A Kaplan, MD
- Professor of Medicine
- University of Connecticut Health Center
- Ronald J Falk, MD
Ronald J Falk, MD
- Allan Brewster Distinguished Professor of Medicine
- Chair, Department of Medicine
- Director, UNC Kidney Center
- Director, Center for Transplant Care
- 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, which can be abbreviated as GPA, and microscopic polyangiitis (MPA) are related systemic vasculitides. Both are associated with antineutrophil cytoplasmic antibodies (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'.)
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.
The initial immunosuppressive therapy of GPA and MPA will be reviewed here. Maintenance immunosuppressive therapy following induction of remission, the treatment of cyclophosphamide-resistant or relapsing disease, the clinical manifestations and diagnosis of these disorders, and patient and renal outcomes are discussed elsewhere. (See "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" 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".)
Assessment of disease activity — A variety of methods have been used to assess disease activity in patients with granulomatosis with polyangiitis, abbreviated as GPA, or microscopic polyangiitis (MPA). As an example, the Birmingham Vasculitis Activity Score (BVAS/GPA) has been applied to patients with either GPA or MPA. The score includes both general symptoms (arthralgia, arthritis, and fever) and involvement of eight major organ systems . (See "Overview of the management of vasculitis in adults", section on 'Monitoring' and "Overview of the management of vasculitis in adults".)
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- NEW TERMINOLOGY
- GENERAL PRINCIPLES
- Assessment of disease activity
- Who should be treated
- Definition of complete remission
- - Renal remission
- - Active disease versus irreversible injury
- - Partial remission
- INITIAL THERAPY
- Overall approach to initial therapy
- Trials comparing cyclophosphamide with rituximab
- Cyclophosphamide-based regimen
- - Daily oral cyclophosphamide
- Oral cyclophosphamide dosing
- Rate and time to remission
- Persistent disease-related morbidity
- - Monthly intravenous cyclophosphamide
- Intravenous cyclophosphamide dosing
- - Cyclophosphamide resistance
- - Contraindications to or refusal of cyclophosphamide
- Rituximab-based regimen
- Glucocorticoid dosing
- Role of plasma exchange
- - Severe active renal disease
- - Concurrent anti-GBM antibodies
- - Pulmonary hemorrhage
- Methotrexate in mild disease
- Treatment-associated toxicity
- - PCP prophylaxis
- MANAGEMENT OF UPPER AIRWAY INVOLVEMENT
- MANAGEMENT IN PREGNANCY
- MAINTENANCE THERAPY
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS
- Background and risk stratification
- Choice of therapy
- Drug dosing