Granulomatosis with polyangiitis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) is a rare form of systemic vasculitis.
Antineutrophil cytoplasmic antibody testing may help with diagnosis, but is not reliable for monitoring disease activity.
Treatment is usually considered in two stages: remission induction and remission maintenance. Patients with active disease are treated with corticosteroids and a second immunosuppressive agent. Disease severity is the principal determinant of the most appropriate additional agent.
With appropriate therapy, patients generally experience prolonged survival with a chronic relapsing course. Most patients develop disease- and/or treatment-related morbidity during the course of their disease.
Long-term management requires careful assessment of disease activity, judicious use of immunosuppressive therapies, and referral to specialised centers with multidisciplinary treatment.

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History & Exam
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Cavitary lung nodules in granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)

Citations

Key Articles

Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res (Hoboken). 2021 Aug;73(8):1088-105.[Abstract][Full Text]
Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764.[Abstract][Full Text]
Stone JH, Merkel PA, Spiera R, et al; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15;363(3):221-32.[Abstract][Full Text]

Other Online Resources

BMJ: plasma exchange and glucocorticoid dosing for patients with ANCA-associated vasculitis: a clinical practice guideline
Vasculitis Clinical Research Consortium

Referenced Articles

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47. Jones RB, Tervaert JW, Hauser T, et al; European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010 Jul 15;363(3):211-20.[Abstract]
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