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Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis

Andre A Kaplan, MD
Ronald J Falk, MD
Section Editors
Gerald B Appel, MD
Fernando C Fervenza, MD, PhD
Deputy Editor
Albert Q Lam, MD


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 [4]. (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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Literature review current through: Nov 2016. | This topic last updated: Wed Aug 27 00:00:00 GMT+00:00 2014.
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