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Treatment-resistant granulomatosis with polyangiitis and microscopic polyangiitis

Author
Ronald J Falk, MD
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editor
John P Forman, MD, MSc

NEW TERMINOLOGY

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].

INTRODUCTION

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, crescentic glomerulonephritis), and have somewhat different clinical features and outcomes (including different patterns of pulmonary involvement and relapse rates) (see "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis", section on 'Clinical presentation'). These entities are closely related to renal-limited necrotizing and pauci-immune necrotizing and crescentic glomerulonephritis.

Cyclophosphamide (or rituximab) in combination with glucocorticoids are usually used for initial immunosuppressive therapy [4,5]. Patients who continue to have active vasculitis, despite at least six months of treatment with one of these optimal initial therapies, have treatment-resistant disease.

Treatment-resistant GPA and MPA will be reviewed here. Initial and maintenance immunosuppressive therapy, the clinical manifestations and diagnosis of these diseases and renal-limited disease, the identification and treatment of relapsing disease, and the prognosis of patients with GPA and MPA are discussed elsewhere. (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Identification and management of relapsing disease in 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".)

IDENTIFICATION OF TREATMENT RESISTANCE

Treatment resistance in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) is defined as the presence of active disease affecting a major organ despite optimal initial immunosuppressive therapy with glucocorticoids plus either cyclophosphamide or rituximab for an adequate period of time (ie, three to six months). (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis".)

                    

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Literature review current through: Jul 2015. | This topic last updated: Jul 6, 2015.
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