Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Andre A Kaplan, MD
Andre A Kaplan, MD
- Professor of Medicine
- University of Connecticut Health Center
- 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 that, along with eosinophilic granulomatosis with polyangiitis (Churg-Strauss), make up the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. Both GPA and MPA are associated with ANCA, have many identical clinical manifestations, have many similar histologic features, 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 for GPA and MPA has two main components: induction of remission with initial immunosuppressive therapy, and maintenance of remission with immunosuppressive therapy for a variable period to prevent relapse.
The initial immunosuppressive therapy of GPA and MPA will be reviewed here (algorithm 1). 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 (GPA) or microscopic polyangiitis (MPA). As an example, the Birmingham Vasculitis Activity Score (BVAS) has been applied to patients with either GPA or MPA. The BVAS system incorporates data on disease manifestations in nine categories (ie, general symptoms such as arthralgia, arthritis, and fever, plus involvement of eight major organ systems) and has been the main outcome measurement tool used in clinical trials of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis . Evaluation of patients using the BVAS system is mostly performed in research studies and less commonly in clinical practice. (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
- - Distinguishing active disease, irreversible injury, and treatment-related damage
- - 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
- - Pulse intravenous cyclophosphamide
- Intravenous cyclophosphamide dosing
- - Cyclophosphamide resistance
- - Contraindications to or refusal of cyclophosphamide
- Rituximab-based regimen
- Glucocorticoid dosing
- Combination cyclophosphamide and rituximab
- Role of plasma exchange
- - Severe active renal disease
- - Concurrent anti-GBM autoantibodies
- - Pulmonary hemorrhage
- Methotrexate in non-organ- and non-life-threatening disease
- Treatment-associated toxicity
- - Prophylaxis against infection
- MANAGEMENT OF UPPER AIRWAY INVOLVEMENT
- MANAGEMENT IN PREGNANCY
- MAINTENANCE THERAPY
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS