Last reviewed: 21 Apr 2022
Last updated: 04 Jun 2019
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)
More key diagnostic factors
Risk factors
- age >50 years
- white ancestry
More risk factors
Diagnostic investigations
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- antineutrophil cytoplasmic auto-antibodies (ANCA)
- blood urea nitrogen and serum creatinine
- urinalysis
- biopsy of affected tissue
More 1st investigations to order
Investigations to consider
- conventional angiography or magnetic resonance angiography (MRA)
- positron emission tomography (PET) scan
More investigations to consider
Treatment algorithm
INITIAL
suspected or confirmed giant cell arteritis
ACUTE
other than giant cell arteritis
ONGOING
resistant to treatment at any stage of therapy
Contributors
Authors
Philip Seo, MD, MHS

Associate Professor of Medicine
Director, Fellowship Program
Division of Rheumatology
Johns Hopkins University School of Medicine
Director
The Johns Hopkins Vasculitis Center
Baltimore
MD
Disclosures
PS declares that he has no competing interests.
Peer reviewers
Megan Clowse, MD, MPH
Assistant Professor of Medicine
Division of Rheumatology and Immunology
Duke University
Durham
NC
Disclosures
MC declares that she has no competing interests.
Bridget Griffiths, MB ChB, MD, FRCP(UK)
Consultant Rheumatologist
Department of Rheumatology
Freeman Hospital
Newcastle Upon Tyne
UK
Disclosures
BG declares that she has no competing interests.
Differentials
- Infective endocarditis
- Hypercoagulability syndromes
- Systemic lupus erythematosus (SLE)
More DifferentialsGuidelines
- EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis
- BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
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