Anti-neutrophil cytoplasmic antibody (ANCA) testing may help with diagnosis, but is not reliable for monitoring disease activity.
Induction of remission was traditionally achieved using cyclophosphamide and high-dose corticosteroids. Methotrexate may effectively substitute for cyclophosphamide in granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) that is not immediately life- or organ-threatening. Remission can be maintained using low-dose corticosteroids and either azathioprine or methotrexate. Rituximab has demonstrated efficacy and safety as an alternative to cyclophosphamide for induction of remission in severe disease, particularly in the setting of disease relapse. However, the optimal timing and dosing for remission maintenance has not been clearly defined, and questions remain regarding the long-term safety profile of rituximab in this population.
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 and judicious use of immunosuppressive therapies, emphasising the importance of referral to, and multidisciplinary treatment in, specialised centres.
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
- white ethnicity
- gastrointestinal involvement
- cardiac involvement
- breast mass
- lower GU tract involvement
- endocrine involvement
- isolated mass lesions/focal granuloma
- genetic predisposition
- environmental exposures
1st investigations to order
- urinalysis and microscopy
- CT chest
- anti-neutrophil cytoplasmic antibody (ANCA)
- FBC and differential
- serum creatinine
- erythrocyte sedimentation rate (ESR)
Investigations to consider
- tissue biopsy
- pulmonary function testing
- electromyography/nerve conduction studies
- upper airway endoscopy
- CT sinuses
non-life/organ-threatening disease excluding isolated subglottic stenosis
isolated subglottic stenosis
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 participated in local advisory boards for Pfizer, Roche, BMS, MSD, and Novartis. He has received conference travel/accommodation expenses from Pfizer, Roche, Abbvie, and MSD. The organisation he works for has an unrestricted educational grant from Roche to support regional educational meetings.
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.
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