Overview
Giant cell arteritis (GCA), also known as temporal arteritis or cranial arteritis, is a systemic inflammatory vasculitis that primarily affects medium-to-large arteries, with a particular predilection for the branches of the external carotid artery, especially the temporal arteries. It is the most common form of systemic vasculitis in adults over the age of 50 in Western countries. The disease is characterized by granulomatous inflammation of the arterial wall, leading to vessel wall thickening, luminal narrowing, and potential occlusion. GCA predominantly affects the vascular system but can have significant consequences for the eyes, brain, and aorta. Key symptoms include new-onset severe headache (often localized to the temporal region), scalp tenderness, jaw claudication (pain with chewing), and visual disturbances that can progress to irreversible vision loss if untreated. Systemic symptoms such as fatigue, fever, weight loss, and malaise are common. Approximately 40-60% of patients with GCA also have polymyalgia rheumatica, characterized by pain and stiffness in the shoulders, neck, and hip girdle. Serious complications include anterior ischemic optic neuropathy leading to permanent blindness, stroke, and aortic aneurysm or dissection. Diagnosis is supported by elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), temporal artery biopsy showing granulomatous inflammation with multinucleated giant cells, and increasingly by imaging modalities such as temporal artery ultrasound or PET-CT. Treatment relies on high-dose glucocorticoids, which should be initiated immediately upon clinical suspicion to prevent vision loss. Tocilizumab, an interleukin-6 receptor inhibitor, has been approved as a steroid-sparing agent and has significantly improved the treatment landscape by reducing relapse rates and cumulative glucocorticoid exposure. Methotrexate may also be used as an adjunctive steroid-sparing therapy. Long-term monitoring for relapse and glucocorticoid-related side effects is essential.
Also known as:
Clinical phenotype terms— hover any for plain English:
Multifactorial
Caused by a mix of several genes and environmental factors
Late onset
Begins later in life, typically after age 50
FDA & Trial Timeline
10 eventsSanofi — PHASE4
University of Edinburgh
Groupe français d'étude des Maladies Inflammatoires de loeil
Hôpital NOVO
Second Affiliated Hospital, School of Medicine, Zhejiang University — NA
Insel Gruppe AG, University Hospital Bern — PHASE3
University Hospital, Brest — PHASE3
National Institute of Allergy and Infectious Diseases (NIAID) — PHASE2
Vastra Gotaland Region
Rigshospitalet, Denmark
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
2 availablePIROXICAM
For relief of the signs and symptoms of osteoarthritis
Actemra
indicated for the treatment of giant cell arteritis (GCA) in adult patients
Rare Disease Specialist
Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
3 resourcesDiclofenac Epolamine
IBSA
Osteoarthritis
Travel Grants
No travel grants are currently matched to Giant cell arteritis.
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Common questions about Giant cell arteritis
What is Giant cell arteritis?
Giant cell arteritis (GCA), also known as temporal arteritis or cranial arteritis, is a systemic inflammatory vasculitis that primarily affects medium-to-large arteries, with a particular predilection for the branches of the external carotid artery, especially the temporal arteries. It is the most common form of systemic vasculitis in adults over the age of 50 in Western countries. The disease is characterized by granulomatous inflammation of the arterial wall, leading to vessel wall thickening, luminal narrowing, and potential occlusion. GCA predominantly affects the vascular system but can h
How is Giant cell arteritis inherited?
Giant cell arteritis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Giant cell arteritis typically begin?
Typical onset of Giant cell arteritis is late onset. Age of onset can vary across affected individuals.
Are there clinical trials for Giant cell arteritis?
Yes — 20 recruiting clinical trials are currently listed for Giant cell arteritis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Giant cell arteritis?
25 specialists and care centers treating Giant cell arteritis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Giant cell arteritis?
1 patient support program are currently tracked on UniteRare for Giant cell arteritis. See the treatments and support programs sections for copay assistance, eligibility, and contact details.