Cranial Arteritis: Symptoms, Types, Causes and Treatment

Cranial arteritis, also known as giant cell arteritis (GCA) or temporal arteritis, is an inflammatory disease of blood vessels that predominantly affects older adults. Because it can lead to severe complications—most notably irreversible vision loss—timely recognition and treatment are critical. This comprehensive guide explores the symptoms, types, underlying causes, and modern treatment options for cranial arteritis, providing an up-to-date, evidence-based resource for patients, caregivers, and healthcare professionals.

Symptoms of Cranial Arteritis

Cranial arteritis is notorious for its diverse and sometimes subtle clinical presentations. Recognizing the symptoms promptly can mean the difference between recovery and life-altering complications such as blindness. While some presentations are classic, others may be atypical and easily overlooked.

Symptom	Description	Prevalence/Notes	Source(s)
Headache	Severe, often temporal	Most common presenting symptom	2 3 5 10
Vision Loss	Sudden, often irreversible	Up to 50% if untreated	2 3 7 10
Jaw Claudication	Pain/fatigue when chewing	Ischemic in origin	2 3 10
Polymyalgia	Aching in shoulders/hips	Often co-occurs with GCA	2 3 8 10
Systemic Symptoms	Fatigue, fever, weight/appetite loss	Nearly universal	3 10
Visual Hallucinations	Formed visual imagery	Rare, newly described	1

Table 1: Key Symptoms of Cranial Arteritis

Classic and Common Symptoms

Headache
The hallmark of cranial arteritis is a new, severe headache, typically localized to the temples. Patients often describe the pain as throbbing or burning, and it may worsen with touch or combing hair. Tenderness of the scalp over the temporal arteries is common, and sometimes the arteries themselves are prominent or nodular on examination 2 3 5 10.

Visual Disturbances
Visual symptoms are among the most feared complications. These can range from transient blurring or double vision to sudden, painless, permanent vision loss. If untreated, up to half of patients may lose vision, usually due to anterior ischemic optic neuropathy. Visual field defects are common, including central scotomas and arcuate or altitudinal losses. Hallucinations, though rare, have also been reported 1 7.

Jaw Claudication
Pain or fatigue in the jaw muscles during chewing is a classic ischemic symptom, highly suggestive of cranial arteritis. It reflects compromised blood flow to the muscles of mastication and is considered a red flag for the disease 2 10.

Polymyalgia Rheumatica Symptoms
Many patients experience muscle pain and stiffness in the shoulders and hips, often worse in the morning. This presentation, known as polymyalgia rheumatica (PMR), may precede, accompany, or follow cranial symptoms. The overlap between PMR and cranial arteritis is well recognized, and both conditions may coexist 2 3 8 10.

Systemic and Atypical Presentations

Systemic Symptoms
Virtually all patients report nonspecific symptoms such as fever, profound fatigue, loss of appetite, and weight loss. These features can be mistaken for infection or malignancy, especially in elderly patients 3 10.

Uncommon Symptoms
In rare cases, patients may experience formed visual hallucinations—distinct, well-structured images not previously reported as a symptom of cranial arteritis 1. Other unusual symptoms can include dizziness, hearing loss, tongue pain, or even stroke if larger vessels are involved 4 9.

Types of Cranial Arteritis

Cranial arteritis is not a uniform disease—rather, it exists along a spectrum, with different clinical phenotypes and varying potential for systemic involvement. Understanding these types helps guide diagnosis and management.

Type	Features	Prevalence/Notes	Source(s)
Classic Cranial GCA	Temporal artery involvement, cranial Sx	Most recognized type	2 3 4 5 6
Large-Vessel GCA	Involvement of aorta & major branches	Increasingly recognized	4 6 9
Polymyalgia Rheumatica	Musculoskeletal pain/stiffness predominant	May occur with/without GCA	3 8 10
Overlapping Phenotypes	Both cranial and systemic/PMR features	Common in clinical practice	3 4 8

Table 2: Types of Cranial Arteritis

Classic Cranial Giant Cell Arteritis

Classic cranial GCA is defined by inflammation of the branches of the external carotid artery, most notably the superficial temporal artery. The classic symptoms—headache, jaw claudication, scalp tenderness, and vision loss—are the result of this localized vasculitis. Temporal artery biopsy is the gold standard for diagnosis in this type 2 3 5 6.

Large-Vessel Giant Cell Arteritis (LV-GCA)

Beyond the cranial arteries, GCA can involve large vessels such as the aorta and its major branches. This phenotype—sometimes called extra-cranial or large-vessel GCA—may present with nonspecific systemic symptoms (fever, malaise, weight loss) and is less likely to have classic cranial findings. Vascular complications (e.g., aneurysms, stenoses, or even stroke) are more common in this group 4 6 9.

Polymyalgia Rheumatica (PMR) and Overlapping Phenotypes

Polymyalgia rheumatica is characterized by bilateral pain and stiffness in the shoulder and pelvic girdles. PMR and GCA are closely linked, and about half of patients with GCA experience PMR symptoms. Some patients may present only with PMR, only with cranial symptoms, or with both—these overlapping phenotypes are common and clinically relevant 3 8 10.

Causes of Cranial Arteritis

The precise cause of cranial arteritis remains elusive, but research points to a combination of age-related, immune, and genetic factors. Understanding the underlying mechanisms is key to recognizing risk and developing effective treatments.

Cause/Factor	Description	Evidence/Notes	Source(s)
Immune Dysregulation	Chronic granulomatous inflammation	Central in pathogenesis	3 4 5 10
Aging	Prevalence increases with age	Mainly affects >50 yrs old	2 3 5 10
Genetic Susceptibility	Familial risk, HLA associations	Some evidence; not decisive	3 5 10
Vascular Predilection	Medium/large arteries affected	Superficial temporal artery	2 4 5
Environmental Triggers	Possible infections/unknown factors	Not well-defined	3 5

Table 3: Causes and Risk Factors for Cranial Arteritis

Immune System Malfunction

Cranial arteritis is fundamentally a disorder of immune dysregulation. The blood vessel wall becomes infiltrated with immune cells, forming granulomas (clusters of macrophages, often with giant cells). These immune attacks cause inflammation, narrowing, and sometimes complete blockage of the arteries, depriving tissues—such as the optic nerve—of blood and leading to symptoms 3 4 5 10.

The Role of Aging

The disease almost exclusively affects individuals over the age of 50, with incidence rising steadily with advancing age. The reasons are not fully understood, but age-related changes in the immune system and blood vessels likely play a central role 2 3 5 10.

Genetics and Susceptibility

Some studies suggest a genetic component, with familial clustering and certain HLA (human leukocyte antigen) types being more common in affected individuals. However, genetic risk is not enough on its own to cause the disease 3 5 10.

Vascular and Environmental Factors

GCA selectively targets medium and large arteries, including the branches of the carotid and sometimes the aorta and its branches. Environmental triggers—potentially infections or other unknown stimuli—may contribute, but no specific cause has been firmly established 2 3 4 5.

Treatment of Cranial Arteritis

Treatment of cranial arteritis is urgent and centers on preventing irreversible complications, particularly vision loss. Recent advances mean more options are available than ever, but timely intervention remains the key to success.

Therapy	Main Use/Purpose	Considerations/Notes	Source(s)
Glucocorticoids	First-line, rapid symptom control	High doses tapered over months/years	2 3 4 7 10
Intravenous Steroids	Severe visual symptoms	May improve prognosis for vision	7 10
Methotrexate	Steroid-sparing, relapse reduction	Modest efficacy; used in relapses	10
Tocilizumab	Biologic, anti-IL-6 therapy	Effective for remission, flare prevention	4 8 10
Other Biologics	For refractory/relapsing cases	Abatacept, ustekinumab, anakinra	10
Endovascular Therapy	Refractory intracranial stenosis	Rare; for severe, resistant cases	9

Table 4: Treatment Strategies for Cranial Arteritis

Glucocorticoids: The Cornerstone

High-dose corticosteroids (prednisone 40–60 mg/day or higher) are the mainstay of therapy. Treatment should begin as soon as cranial arteritis is suspected—delays can result in irreversible complications. Most patients experience rapid symptom improvement. Steroid therapy is usually tapered slowly over 6 months to 2 years, with regular monitoring for relapse 2 3 4 10.

• Intravenous methylprednisolone is sometimes used for patients with acute vision loss or severe symptoms, and may offer a better prognosis for visual recovery compared to oral steroids alone 7 10.
• Despite prompt treatment, some patients may still lose vision, and relapses are not uncommon as steroids are tapered 7 10.

Steroid-Sparing and Adjunctive Therapies

Methotrexate is the most studied immunosuppressant used alongside steroids. While it can help reduce relapses and steroid exposure, its efficacy is modest, and side effects must be considered 10.

Tocilizumab, a biologic agent targeting the interleukin-6 receptor, has emerged as a highly effective steroid-sparing therapy. It reduces the risk of flares, helps achieve sustained remission, and lowers cumulative steroid doses. Tocilizumab is effective for all clinical phenotypes—cranial, PMR, or overlapping 4 8 10.

Other biologics, such as abatacept, ustekinumab, and anakinra, are being investigated for use in refractory or relapsing disease, but more research is needed 10.

Advanced and Rescue Therapies

In rare, severe cases—especially when intracranial arteries are affected and standard therapies fail—endovascular treatments such as percutaneous transluminal angioplasty (PTA), sometimes with stenting, may be considered. These interventions are experimental and reserved for select patients with medically refractory disease 9.

Monitoring and Follow-up

Long-term monitoring is essential to:

• Detect relapses early
• Manage side effects of long-term steroids and immunosuppressants
• Screen for large-vessel complications (aneurysms, stenoses)
• Adjust therapy as needed 3 4 10

Conclusion

Cranial arteritis is a complex, potentially devastating disease that primarily affects older adults. Early recognition and aggressive treatment are vital to prevent irreversible complications, especially blindness. Advances in biologic therapies and improved imaging have transformed management and outcomes, but challenges remain.

Key takeaways:

• Symptoms are diverse—classic features include headache, vision loss, jaw claudication, and polymyalgia, but atypical presentations exist.
• Multiple types exist, ranging from classic cranial to large-vessel and overlapping PMR phenotypes.
• Underlying causes involve immune dysfunction, aging, genetic susceptibility, and perhaps environmental factors.
• Treatment is urgent and multi-modal, with glucocorticoids as first-line therapy and biologic agents like tocilizumab as promising steroid-sparing options.
• Long-term follow-up is essential to monitor for relapses and complications.

Stay alert to the signs—early intervention saves sight and lives.