Last reviewed: 12 Nov 2024
Last updated: 29 Nov 2023
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)
Full details
Risk factors
- extremes of age
Full details
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
Full details
Tests to consider
- conventional angiography or magnetic resonance angiography (MRA)
- positron emission tomography (PET) scan
Full details
Treatment algorithm
INITIAL
suspected giant cell arteritis
ACUTE
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)
ONGOING
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.
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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