Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a rare form of systemic vasculitis.
Anti-neutrophil 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 centres with multidisciplinary treatment.
Granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) is a systemic vasculitis that typically involves small and medium vessels. Although any organ may be targeted, the classic triad consists of upper and lower respiratory tract involvement and pauci-immune glomerulonephritis. Involvement of cutaneous, ocular, musculoskeletal, and peripheral nervous system tissue is also common.
History and exam
Key diagnostic factors
- upper and lower respiratory tract involvement
- renal involvement
- constitutional features
Other diagnostic factors
- ocular manifestations
- cutaneous manifestations
- musculoskeletal manifestations
- neurological manifestations
- signs or symptoms of thromboembolism
- gastrointestinal involvement
- cardiac involvement
- breast mass
- lower genitourinary tract involvement
- endocrine involvement
- isolated mass lesions/focal granuloma
- genetic predisposition
- environmental exposures
- white ethnicity
1st investigations to order
- urinalysis and microscopy
- CT chest
- anti-neutrophil cytoplasmic antibody (ANCA)
- FBC and differential
- serum creatinine
- C-reactive protein
- erythrocyte sedimentation rate (ESR)
- liver function tests
- serum calcium
Investigations to consider
- tissue biopsy
- pulmonary function testing
- electromyography/nerve conduction studies
- upper airway endoscopy
- CT sinuses
severe (life/organ-threatening) disease: remission induction
non-severe (non-life/organ-threatening) disease: remission induction
remission successfully induced
relapse following successful remission
Eamonn Molloy, MD, MS, FRCPI
Department of Rheumatology
St Vincent's University Hospital
EM declares that he has been a speaker for, and participated in advisory board for Abbvie, Janssen, Gilead, Novartis. He has received sponsorship to attend conferences from Abbvie, Janssen and UCB, and received research funding from Abbvie.
Curry L. Koening, MD, MS
Assistant Professor of Medicine
University of Utah School of Medicine
Salt Lake City
CLK declares that he has no competing interests.
Jaap M. van Laar, MD, PhD
Professor of Clinical Rheumatology
Musculoskeletal Research Group
Institute of Cellular Medicine
The Medical School
Newcastle upon Tyne
JMVL declares that he has no competing interests.
- Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome)
- Microscopic polyangiitis (MPA)
- Classic polyarteritis nodosa (cPAN)
- 2022 American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal disease
- 2022 ACR recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis
- Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer