Temporal Arteritis: Age-Related Blood Vessel Inflammation Affecting Vision

Essential Insights for Temporal Arteritis Management

Table of Contents

• Understanding Temporal Arteritis and Giant Cell Arteritis
• Early Warning Signs and Symptoms of Temporal Arteritis
• How Does Temporal Arteritis Affect Vision and Eye Health?
• Diagnosing Temporal Arteritis: Tests and Procedures
• Can Temporal Arteritis Cause Permanent Blindness?
• Treatment Options to Prevent Vision Loss in Elderly Patients
• The Connection Between Polymyalgia Rheumatica and Arteritis
• Managing Long-Term Care for Patients with Temporal Arteritis

Understanding Temporal Arteritis and Giant Cell Arteritis

Temporal arteritis, also known as giant cell arteritis (GCA), is a serious inflammatory condition affecting the medium and large arteries, particularly those in the head and neck. This condition predominantly affects adults over 50 years of age, with the risk increasing significantly with each decade of life. Women are approximately three times more likely to develop temporal arteritis than men, and it is more common in individuals of Northern European descent.

At its core, temporal arteritis is an autoimmune disorder where the body’s immune system mistakenly attacks the lining of arteries, causing inflammation that leads to narrowing and potential blockage. The name “temporal arteritis” derives from the frequent involvement of the temporal arteries, which run along the temples on either side of the head. However, the condition can affect multiple arteries throughout the body, which is why the term “giant cell arteritis” is often preferred by medical professionals as it more accurately reflects the widespread nature of the disease.

The inflammation in the arterial walls is characterised by the presence of giant cells (large multinucleated cells formed by the fusion of several macrophages) visible under microscopic examination. This distinctive histological feature gives the condition its alternative name. The inflammatory process causes the arterial walls to thicken, reducing blood flow to vital structures, including the eyes and brain, which can lead to serious complications if left untreated.

Early Warning Signs and Symptoms of Temporal Arteritis

Recognising the early warning signs of temporal arteritis is crucial for timely intervention and prevention of serious complications, particularly vision loss. The symptoms of temporal arteritis can vary significantly between individuals, but several characteristic manifestations should prompt immediate medical attention.

Headache is often the most prominent initial symptom, typically described as severe, persistent, and localised to the temples or the back of the head. Unlike ordinary headaches, the pain associated with temporal arteritis tends to be more intense and may not respond well to over-the-counter pain relievers. Many patients report tenderness of the scalp, particularly over the temporal arteries, which may appear prominent, nodular, or erythematous upon examination.

Jaw claudication—pain in the jaw muscles when chewing or talking—is a highly specific symptom that occurs in approximately 40-50% of patients with temporal arteritis. This discomfort typically worsens with prolonged chewing and improves with rest. Systemic symptoms are also common and include unexplained fever, fatigue, malaise, and unintentional weight loss, reflecting the systemic inflammatory nature of the condition.

Cranial nerve symptoms may develop as the inflammation affects the blood supply to these important structures. Patients might experience double vision (diplopia), facial pain, or hearing difficulties. Additionally, some individuals report prominent shoulder and hip pain, which may indicate the presence of polymyalgia rheumatica, a related inflammatory condition that frequently coexists with temporal arteritis. Any sudden visual disturbances, including blurred vision, vision loss in one eye, or transient visual obscurations, represent a medical emergency and require immediate assessment to prevent permanent blindness.

How Does Temporal Arteritis Affect Vision and Eye Health?

Temporal arteritis poses a significant threat to vision and eye health through its effects on the blood vessels supplying crucial ocular structures. The inflammatory process primarily affects the posterior ciliary arteries, which provide blood to the optic nerve head, and the ophthalmic artery, the main arterial supply to the eye. When these vessels become inflamed and narrowed, the resulting ischaemia (inadequate blood supply) can lead to devastating visual consequences.

The most serious ocular complication of temporal arteritis is anterior ischaemic optic neuropathy (AION), which occurs when inflammation restricts blood flow to the anterior portion of the optic nerve. This results in sudden, painless vision loss that can progress rapidly. Unlike non-arteritic AION, which typically affects one eye, the arteritic form associated with temporal arteritis can affect both eyes sequentially, often within days or weeks if left untreated.

Other visual manifestations may include central retinal artery occlusion, which causes sudden and complete vision loss in the affected eye, and posterior ischaemic optic neuropathy, affecting the posterior portion of the optic nerve. Some patients experience amaurosis fugax—transient episodes of monocular vision loss lasting minutes to hours—which may serve as a warning sign before permanent vision loss occurs.

The visual symptoms of temporal arteritis can sometimes be subtle initially, with patients reporting blurred vision, visual field defects, or diplopia (double vision) due to ischaemia affecting the extraocular muscles or their nerve supply. Importantly, once vision loss occurs due to temporal arteritis, it is often irreversible, highlighting the critical importance of early diagnosis and prompt treatment to preserve visual function.

Diagnosing Temporal Arteritis: Tests and Procedures

Diagnosing temporal arteritis requires a comprehensive approach combining clinical assessment, laboratory investigations, and often histopathological confirmation. Given the potential for rapid vision loss, clinicians must maintain a high index of suspicion in elderly patients presenting with suggestive symptoms.

Laboratory tests play a crucial role in the initial evaluation. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are inflammatory markers typically elevated in temporal arteritis. An ESR greater than 50 mm/hour in patients over 50 years old is highly suggestive, though not diagnostic. These tests are also valuable for monitoring disease activity during treatment. Complete blood count may reveal normochromic, normocytic anaemia, reflecting chronic inflammation.

Temporal artery biopsy remains the gold standard for definitive diagnosis. This outpatient procedure involves removing a 2-3 cm segment of the temporal artery under local anaesthesia for histopathological examination. The characteristic findings include granulomatous inflammation with multinucleated giant cells and fragmentation of the internal elastic lamina. Since the inflammatory changes can be segmental (skip lesions), a negative biopsy does not completely exclude the diagnosis if clinical suspicion remains high.

Advanced imaging modalities have emerged as valuable adjunctive diagnostic tools. Colour Doppler ultrasonography can demonstrate the “halo sign”—a hypoechoic ring around the temporal artery lumen representing vessel wall oedema. High-resolution magnetic resonance imaging (MRI) may show arterial wall thickening and enhancement, while positron emission tomography (PET) can identify widespread vascular inflammation, particularly useful in cases with atypical presentations or inconclusive biopsies. These imaging techniques are increasingly used to guide biopsy site selection and may help diagnose cases where biopsy is contraindicated or declined.

Can Temporal Arteritis Cause Permanent Blindness?

Temporal arteritis represents one of the few true ophthalmic emergencies, as it can indeed cause permanent blindness if not promptly diagnosed and treated. The risk of vision loss in untreated temporal arteritis is substantial, with studies suggesting that up to 15-20% of patients may develop permanent visual impairment. This risk increases dramatically with delayed treatment, highlighting the critical importance of early intervention.

The mechanism of vision loss in temporal arteritis involves ischaemic damage to the optic nerve and other ocular structures due to inflammation-induced narrowing of the supplying arteries. Once this ischaemic damage occurs, the resulting vision loss is typically irreversible, even with subsequent treatment. The optic nerve has limited capacity for regeneration, and the death of retinal ganglion cells and their axons is permanent.

The progression to blindness can be alarmingly rapid. In some cases, patients may experience complete vision loss within hours to days after the onset of visual symptoms. More concerning is that approximately 25-50% of patients who experience vision loss in one eye will develop involvement in the second eye within 1-2 weeks if left untreated, potentially resulting in complete blindness.

Certain clinical features may indicate a higher risk of vision loss, including advanced age, male gender, a recent history of amaurosis fugax (transient monocular vision loss), and the presence of jaw claudication. Notably, some patients may develop vision loss with minimal or no preceding systemic symptoms, making temporal arteritis a challenging diagnosis. This underscores the importance of considering temporal arteritis in any elderly patient presenting with new-onset visual symptoms, even in the absence of classic systemic manifestations such as headache or scalp tenderness.

Treatment Options to Prevent Vision Loss in Elderly Patients

The cornerstone of temporal arteritis treatment is high-dose corticosteroid therapy, which should be initiated immediately upon strong clinical suspicion, even before confirmatory tests are completed. This urgency is justified by the potential for rapid, irreversible vision loss if treatment is delayed. The standard initial therapy involves prednisolone at doses of 40-60 mg daily (or equivalent), which effectively suppresses the inflammatory process in most patients.

For patients presenting with visual symptoms or at high risk of vision loss, intravenous methylprednisolone (500-1000 mg daily for 3 days) may be administered before transitioning to oral therapy. This approach provides rapid anti-inflammatory effects and may offer additional protection against vision loss, though evidence for superior efficacy compared to high-dose oral therapy remains limited.

Once clinical improvement is evident and inflammatory markers normalise (typically within 2-4 weeks), a carefully monitored steroid taper can begin. This tapering process is typically slow, often extending over 12-24 months, with dose adjustments guided by clinical symptoms and laboratory parameters. Premature reduction of corticosteroids significantly increases the risk of disease relapse and vision loss.

Given the prolonged course of corticosteroid therapy and its associated side effects—including osteoporosis, diabetes, hypertension, and increased infection risk—adjunctive treatments are essential. Bone protection with calcium, vitamin D supplements, and bisphosphonates should be initiated concurrently with corticosteroids. For patients unable to tolerate corticosteroids or requiring steroid-sparing strategies, immunosuppressive agents such as methotrexate, leflunomide, or tocilizumab (an IL-6 receptor antagonist) may be considered. Recent clinical trials have demonstrated that tocilizumab, in particular, can facilitate more rapid steroid tapering while maintaining disease control, potentially reducing steroid-related complications in elderly patients.

The Connection Between Polymyalgia Rheumatica and Arteritis

Polymyalgia rheumatica (PMR) and temporal arteritis share a profound clinical relationship, with significant overlap in their pathophysiology, demographic profile, and management approaches. PMR is characterised by bilateral pain and stiffness in the shoulder and hip girdles, particularly pronounced in the morning or after periods of inactivity. Like temporal arteritis, PMR predominantly affects individuals over 50 years of age and is more common in women and those of Northern European descent.

The connection between these conditions is substantial—approximately 40-60% of patients with temporal arteritis concurrently have PMR, while about 15-30% of patients initially diagnosed with PMR will develop temporal arteritis during their disease course. This bidirectional relationship suggests shared pathogenic mechanisms, likely involving similar genetic predispositions and immunological pathways. Both conditions demonstrate elevated inflammatory markers, including ESR and CRP, and respond to corticosteroid therapy, albeit at different dosage requirements.

From a clinical perspective, the presence of PMR symptoms in an elderly patient should prompt vigilance for temporal arteritis. Conversely, patients diagnosed with PMR who develop new headaches, jaw claudication, visual disturbances, or other cranial symptoms require immediate evaluation for temporal arteritis. The typical corticosteroid dose required for PMR (15-20 mg prednisolone daily) is insufficient to prevent vision loss in temporal arteritis, underscoring the importance of distinguishing between these conditions.

Recent research has identified shared genetic susceptibility loci, particularly in the HLA region, and similar patterns of cytokine dysregulation in both conditions. Imaging studies using PET scans have demonstrated that many patients with apparent isolated PMR actually have subclinical large vessel vasculitis, suggesting these conditions may represent different manifestations of a spectrum of disease rather than entirely separate entities. This evolving understanding has implications for monitoring strategies and treatment decisions, particularly regarding the duration of immunosuppressive therapy and the vigilance required during treatment tapering.

Managing Long-Term Care for Patients with Temporal Arteritis

Long-term management of temporal arteritis requires a carefully structured approach that balances disease control with minimisation of treatment-related complications. Most patients require corticosteroid therapy for 1-2 years, though some may need treatment for 5 years or longer. The tapering schedule must be individualised, with typical reductions of 10% of the current dose every 2-4 weeks once the disease is stabilised, and more gradual reductions at lower doses.

Regular monitoring is essential throughout the disease course. Clinical assessments should evaluate for symptom recurrence, while laboratory monitoring of inflammatory markers (ESR and CRP) helps detect subclinical disease activity. However, age-related increases in these markers and their potential elevation due to concurrent conditions necessitate interpretation in the clinical context rather than as absolute indicators. Some centres employ vascular imaging techniques such as ultrasound or PET scanning to assess disease activity in ambiguous cases.

Comprehensive management must address the substantial burden of corticosteroid-related adverse effects in this predominantly elderly population. Regular assessments of bone health, including DEXA scans, are recommended, along with monitoring for diabetes, hypertension, cataracts, and glaucoma. Psychological effects of corticosteroids, including mood disturbances and sleep disruption, should not be overlooked. Proactive management of these complications significantly improves quality of life and treatment adherence.

Multidisciplinary care involving rheumatologists, ophthalmologists, and primary care physicians provides optimal management. Ophthalmological follow-up is particularly important for patients who have experienced visual symptoms, as they may benefit from low vision services and rehabilitation if permanent visual impairment has occurred. Patient education regarding the importance of treatment adherence, potential disease flares, and when to seek urgent medical attention remains crucial throughout the disease course. Support groups and patient resources can provide valuable additional assistance, helping patients navigate the challenges of living with a chronic inflammatory condition while managing the effects of long-term immunosuppressive therapy.

Frequently Asked Questions

What are the first signs of temporal arteritis?

The first signs of temporal arteritis typically include severe headaches localized to the temples, scalp tenderness, jaw pain when chewing (jaw claudication), and fatigue. Some patients may experience fever, unexplained weight loss, and shoulder/hip pain. Visual disturbances such as blurred vision or temporary vision loss are serious warning signs that require immediate medical attention as they may precede permanent blindness.

How quickly can temporal arteritis cause blindness?

Temporal arteritis can cause blindness rapidly—sometimes within hours to days after the onset of visual symptoms. Without treatment, approximately 15-20% of patients develop permanent visual impairment. More concerning, 25-50% of patients who lose vision in one eye may develop involvement in the second eye within 1-2 weeks if left untreated, potentially resulting in complete blindness.

Is temporal arteritis considered a medical emergency?

Yes, temporal arteritis is considered a true ophthalmic emergency. The condition requires immediate medical attention and prompt initiation of high-dose corticosteroid therapy, even before confirmatory tests are completed. This urgency is justified by the risk of rapid, irreversible vision loss if treatment is delayed. Any elderly person with suspicious symptoms should seek emergency care.

What is the relationship between polymyalgia rheumatica and temporal arteritis?

Polymyalgia rheumatica (PMR) and temporal arteritis have a strong clinical relationship. Approximately 40-60% of patients with temporal arteritis concurrently have PMR, while about 15-30% of patients initially diagnosed with PMR will develop temporal arteritis. Both conditions affect similar demographics, share genetic susceptibility factors, and respond to corticosteroid therapy, though temporal arteritis requires higher doses for treatment.

How is temporal arteritis diagnosed?

Temporal arteritis is diagnosed through a combination of clinical assessment, laboratory tests, and confirmatory procedures. Key diagnostic elements include elevated inflammatory markers (ESR and CRP), temporal artery biopsy showing characteristic inflammatory changes, and imaging studies such as ultrasound, MRI, or PET scans. Temporal artery biopsy remains the gold standard, though imaging techniques are increasingly used as complementary diagnostic tools.

How long does treatment for temporal arteritis typically last?

Treatment for temporal arteritis typically lasts 1-2 years, though some patients may require therapy for 5 years or longer. The initial high-dose corticosteroid treatment (40-60mg prednisolone daily) is gradually tapered once symptoms improve and inflammatory markers normalize. The tapering process is slow, with dose reductions of approximately 10% every 2-4 weeks, becoming more gradual at lower doses to prevent disease relapse.