Summary
Definition
History and exam
Key diagnostic factors
- constitutional symptoms (e.g., malaise, fever, arthralgia, myalgia)
- headache and scalp tenderness (giant cell arteritis)
- visual changes (large-vessel)
- upper extremity or jaw claudication (large-vessel)
- asymmetric brachial pulses (large-vessel)
- bruits (large-vessel)
- abdominal pain (medium-vessel)
- foot drop, wrist drop (medium-vessel)
- cutaneous ulcers (medium-vessel)
- hematuria (small-vessel)
- palpable purpura (small-vessel)
- otorrhea, ear pain, or muffled sensation in the ears (small-vessel)
- nasal symptoms (small-vessel)
- sinus pain (small-vessel)
- wheeze (small-vessel)
- hemoptysis (small-vessel)
Risk factors
- extremes of age
Diagnostic tests
1st tests to order
- erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- antineutrophil cytoplasmic autoantibodies (ANCA)
- assessment for glomerulonephritis
- biopsy of affected tissue
Tests to consider
- conventional angiography or magnetic resonance angiography (MRA)
- positron emission tomography (PET) scan
Treatment algorithm
suspected giant cell arteritis
life-threatening or organ-threatening granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)
non-life-threatening, non-organ-threatening granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)
resistant to treatment at any stage of therapy
Contributors
Authors
Brendan Antiochos, MD
Assistant Professor of Medicine
Director, Vasculitis Center
Division of Rheumatology
Johns Hopkins
Baltimore
MD
Disclosures
BA has received compensation from Paradigm Medical Communications for CME activities regarding EGPA and Giant Cell Arteritis.
Acknowledgements
Dr Brendan Antiochos would like to gratefully acknowledge Dr Phillip Seo, the previous contributor for this topic.
Disclosures
PS is an author of some references cited in this topic.
Peer reviewers
Jason M. Springer, MD, MS
Associate Professor
Vanderbilt University Medical Center
Nashville
TN
Disclosures
JMS has served as a consultant and on an advisory board for ChemoCentryx, the manufacturer of avacopan.
References
Key articles
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;2022-223764.Full text Abstract
Hellmich B, Agueda A, Monti S, et al. 2018 update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30.Full text Abstract
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 Rheumatol. 2021 Aug;73(8):1366-83.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Infective endocarditis
- Hypercoagulability syndromes
- Systemic lupus erythematosus (SLE)
More DifferentialsGuidelines
- American College of Rheumatology guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
- EULAR recommendations for the management of ANCA-associated vasculitis
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