eMedicine Specialties > Emergency Medicine > Rheumatology

Temporal Arteritis

Christopher H Lee, MD, Clinical Instructor, Section of EMS, Department of Emergency Medicine, Yale University School of Medicine
Jean Marie Hammel, MD, Assistant Professor, Associate Residency Director of Emergency Medicine Residency Program, Department of Surgery, Section of Emergency Medicine, Yale University School of Medicine

Updated: Sep 8, 2009

Introduction

Background

Temporal arteritis (TA), also known as giant cell arteritis (GCA), is a common form of systemic vasculopathy affecting patients older than 50 years. Although typically affecting the superficial temporal arteries, this inflammatory process has been shown to involve medium- and large-sized vessels, including the aorta, carotid, subclavian, vertebral, and iliac arteries. Therefore, "giant cell arteritis" may be more appropriate than "temporal arteritis" to identify this type of vasculitis, though both terms are used interchangeably.

Hematoxylin and eosin stained femoral artery branch, cross section, taken from a lower limb amputation specimen from the same patient shown in Media files 1-2. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from giant cell arteritis resulted in ischemic necrosis of the lower limb, necessitating amputation.

Pathophysiology

The exact etiology of this disease remains unknown. Temporal arteritis is a chronic, systemic vasculitis primarily affecting the elastic lamina of medium- and large-sized arteries. Histopathology of affected arteries is marked by transmural inflammation of the intima, media, and adventitia, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells. Mural hyperplasia can result in arterial luminal narrowing, resulting in subsequent distal ischemia.
  
The temporal artery is commonly affected, often resulting in temporal-lobe headaches. Other commonly affected vessels include the ophthalmic, posterior ciliary, and, to a lesser extent, the central retinal artery. Inflammation in these locations can cause irreversible visual impairment and ischemic optic neuritis.   

Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Although many infectious pathogens, such as Parvovirus B19 and Chlamydia species , have been suggested as possible inciting agents, the actual role of microbial pathogens is still unclear.
  
Current theory regarding the etiology of temporal arteritis holds that a maladaptive response to endothelial injury leads to an inappropriate activation of cell-mediated immunity via immature antigen-presenting cells. The subsequent release of cytokines within the arterial vessel wall can attract macrophages and multinucleated giant cells, which gives diseased vessels their characteristic histology. This also leads to an oligoclonal expansion of T-cells directed against antigens in or near the elastic lamina. Ultimately, this cascade results in vessel wall damage, intimal hyperplasia, and eventual stenotic occlusion.

These inflammatory changes are also seen in polymyalgia rheumatica (PMR). Polymyalgia rheumatica and temporal arteritis are closely related inflammatory conditions, and it is suggested that they may be slightly different manifestations of the same underlying disease process. The symptoms of polymyalgia rheumatica are more systemic, including pain and stiffness in the shoulder and pelvic musculature, as well as fever, malaise, and weight loss. The relationship between polymyalgia rheumatica and temporal arteritis warrants consideration as it has been estimated that approximately half of patients initially presenting with temporal arteritis have been found to also have polymyalgia rheumatica. Conversely, about 10% of patients initially presenting with polymyalgia rheumatica were found to have temporal arteritis upon further investigation.  

The etiology of temporal arteritis is multifactorial, as both genetic and environmental associations have been identified. Some major histocompatibility complex molecules, particularly human leukocyte antigen HLA-DR4 and HLA-DRB104 alleles, may have a role in a patient’s susceptibility to temporal arteritis. Additionally, there is a statistically significant increase in the incidence of temporal arteritis in northern latitudes.

Frequency

United States

Temporal arteritis is typically seen in female patients older than 50 years. Incidence increases with age and can range from 1 in 10,000 to 5 in 10,000.

International

Rates are significantly higher in northern latitudes.

Mortality/Morbidity

Temporal arteritis does not appear to affect long-term survival.
            
Permanent visual loss is the most devastating consequence of temporal arteritis. The incidence of ocular involvement varies greatly in the literature, ranging from 8-50%. Bilateral visual loss can occur in up to 33% of patients.  
 
Rarely, patients can experience neurologic manifestations such as transient ischemic attacks (TIAs) or cerebral vascular accidents (CVAs)¹ . However, the exact relationship between temporal arteritis and TIA/CVA is uncertain.

Race

Temporal arteritis occurs more frequently in white patients, especially those of northern European descent.

Sex

Women develop temporal arteritis 2-3 times more frequently than men.

Age

Temporal arteritis rarely occurs in patients younger than 50 years. The mean age of onset is 72 years. Incidence of the disease increases significantly with increasing age.

Clinical

History

Headache is the most common chief complaint and presents in over two thirds of patients with temporal arteritis. The headache tends to be new or different in character than previous headaches and is typically sudden in onset, localizing to the temporal region. However, pain with temporal arteritis can occur diffusely through the occipital, frontal, or parietal regions as well. Therefore, any new headache in patients older than 50 years warrants a consideration of temporal arteritis.  

Because temporal arteritis tends to affect the branches of the carotid artery, clinical manifestations vary depending on the distribution of the ischemic vessel. For example, superficial temporal artery involvement can lead to severe scalp tenderness during such simple acts as resting the head on a pillow, combing hair, or wearing hats and eyeglasses. Patients may also present with visible areas of scalp necrosis. Similarly, jaw claudication while speaking or chewing is observed in patients with involvement of the maxillary artery, which can occur in half of patients with temporal arteritis.
  
Visual loss may also be a presenting symptom and can be sudden and painless. Initial visual symptoms are usually transient and intermittent, typically manifesting as unilateral visual loss or occasionally diplopia. However, if left untreated, permanent blindness frequently results.  

Constitutional symptoms due to systemic inflammation are common. These nonspecific symptoms include fever, malaise, memory impairment, anorexia, weight loss, fatigue, and depression. Additionally, polymyalgia rheumatica symptoms are present in about half of all cases of temporal arteritis and may be the initial complaint in many patients.
  
Based on the 1990 American College of Rheumatology criteria for classification of temporal arteritis, at least 3 of the following 5 items must be present (sensitivity 93.5%, specificity 91.2%)² : 

1. Age of onset older than 50 years
2. New-onset headache or localized head pain
3. Temporal artery tenderness to palpation or reduced pulsation
4. Erythrocyte sedimentation rate (ESR) greater than 50 mm/h
5. Abnormal arterial biopsy (necrotizing vasculitis with granulomatous proliferation and infiltration)

Physical

A thorough physical and neurological examination should be performed to exclude other possible causes of headache and visual disturbances.

The head and face should be examined for inflamed and thickened arteries, tenderness to palpation, tender scalp nodules, or necrotic areas of the scalp. Inflamed vessels may be tender and warm. They may also appear thickened and dilated, such that the examiner may be able to roll the artery between the fingers and the skull. Cranial nerve palsies, particularly of the sixth nerve, should also be noted.

A complete eye examination should be performed, including visual acuity, visual field check, and funduscopic as well as a slit lamp examinations. Special attention should be paid to the retinal vessels, as other causes of loss of vision such as central retinal artery or vein occlusion can cause a markedly abnormal funduscopic examination result. In temporal arteritis, the funduscopic examination result may be normal or can show dilated retinal veins.

Differential Diagnoses

Workup

Laboratory Studies

• Erythrocyte sedimentation rate (ESR) is a nonspecific marker of inflammation. It is the most commonly used laboratory test in diagnosing temporal arteritis. Most patients with temporal arteritis have an ESR greater than 80 mm/h. However, up to 20% of patients with temporal arteritis may have a normal or low ESR, and thus a normal ESR level can not exclude a diagnosis of temporal arteritis.  
  • Normal ESR levels vary according to a patient’s age and sex. A general guide for estimating normal ESR values uses the following formulas:
    • Males: (0.5 X age)
    • Females: (0.5 X age) + 5      
• C-reactive protein (CRP) is an acute-phase protein released by hepatocytes in inflammatory states. 8CRP has been found to be elevated (>2.45 mg/dL) in patients with temporal arteritis, even in patients with a normal ESR. An advantage to CRP is that the normal value range does not vary with age or sex. A normal CRP is less than 0.5 mg/dL. An elevated CRP may help to make the diagnosis when taken under consideration with a normal ESR.
• Complete blood cell count (CBC) may reveal leukocytosis, anemia, or thrombocytosis. Several studies have documented an association between an elevated platelet count >400 X 10³/L and temporal arteritis. However, this test is not sufficiently sensitive or specific to be useful in the diagnosis of temporal arteritis.
• Elevated liver function test (LFT) results, particularly alkaline phosphatase, are obtained in about one half of patients with temporal arteritis

Imaging Studies

• Color duplex sonography of temporal arteries can be used prior to biopsy for optimal results.³  A sonographic halo sign around temporal arteries may be pathognomonic for temporal arteritis. However, whether ultrasonographic imaging can replace temporal artery biopsy as the definitive diagnostic procedure remains to be seen.

Procedures

• Definitive diagnosis relies on temporal artery biopsy. Biopsy should be performed as an outpatient procedure within 1 week after the initiation of corticosteroid therapy in the emergency department. Although prompt follow-up is optimal, biopsy results have been useful even 3-4 weeks after the initiation of steroid therapy. Since affected areas of vessels can be patchy or segmental, multiple biopsy sites may be required. If clinical suspicion remains high after an initial negative biopsy result, bilateral biopsies may be required.

Hematoxylin and eosin stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of giant cell arteritis shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.

Treatment

Prehospital Care

Patients generally do not present via emergency medical services, and no particular prehospital interventions are warranted.

Emergency Department Care

Optimal care of patients with temporal arteritis in the emergency department involves maintaining a high index of suspicion and a low threshold to treat.  

Treatment consists of corticosteroids. Although corticosteroids are the only proven treatment of temporal arteritis, few studies exist regarding dosing protocols. It is generally agreed that patients with suspected temporal arteritis should be started on oral prednisone 60 mg/day in the emergency department, with a temporal artery biopsy performed as an outpatient procedure scheduled within 1 week.

Improvement of systemic symptoms typically occurs within 72 hours of initiation of therapy. Patients should be counseled that corticosteroid therapy may be lengthy (1-2 y) and can lead to the typical complications associated with long-term steroid use. Recent data suggest that initial high-dose intravenous corticosteroid administration is beneficial in reducing temporal arteritis remission rates.⁴  However, further study is warranted before this is routinely practiced.

Consultations

An ophthalmologist should be consulted for a complete, dilated ocular examination to rule out other causes of vision loss, particularly when the diagnosis is uncertain.  

A rheumatologist or internist should direct follow-up care for these patients, monitor remissions and recurrence, and manage complications associated with long-term corticosteroid therapy.

Medication

Systemic steroids should be started. Oral steroids are effective. Intravenous steroids may be administered if visual deficit is established or if the patient requires admission for other reasons.

Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects.

Prednisone (Deltasone, Sterapred, Orasone)

DOC in treatment of temporal arteritis. Useful in treatment of inflammatory and allergic reactions by reversing increased capillary permeability and suppressing PMN activity.
In prolonged treatment, taper over 1-2 wk.

Dosing

Adult

1-2 mg/kg/d PO

Pediatric

4-5 mg/m²/d
Alternatively, administer 1-2 mg/kg PO qd

Interactions

Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral, fungal, tubercular skin, or connective tissue infections; peptic ulcer disease; hepatic dysfunction; GI disease; use with caution in patients with diabetes

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Methylprednisolone (Solu-Medrol, Medrol, Depo-Medrol)

Decreases inflammation by suppressing migration of PMNs and reversing increased capillary permeability.

Dosing

Adult

125-250 mg IV loading dose, followed by 0.5-1 mg/kg/dose q6h, not to exceed 3 d

Pediatric

2 mg/kg IV loading dose, followed by 0.5-1 mg/kg/dose q6h, not to exceed 3 d

Interactions

Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels of methylprednisolone; phenobarbital, phenytoin, and rifampin may decrease levels of methylprednisolone (adjust dose); monitor patients for hypokalemia when taking medication concurrently with diuretics

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular skin infections; use with care in patients with diabetes

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use

Immunosuppressants

These agents suppress key factors of the immune system involved in immune reactions.

Azathioprine (Imuran)

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

Dosing

Adult

1 mg/kg/d PO qd for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d

Pediatric

Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV

Interactions

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of MTX metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

Contraindications

Documented hypersensitivity; low levels of serum thiopurine methyl transferase (TPMT)

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level prior to therapy and monitor liver, renal, and hematologic function; pancreatitis rarely associated

Methotrexate (Rheumatrex)

Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness).
Adjust dose gradually to attain satisfactory response.

Dosing

Adult

0.3 mg/kg/wk PO/IM; not to exceed 20 mg

Pediatric

Not established

Interactions

Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX
Probenecid, NSAIDs, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, can increase MTX plasma levels; may decrease phenytoin plasma levels; may increase plasma levels of thiopurines

Contraindications

Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency

Precautions

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Monitor CBCs monthly and monitor liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs; fatal reactions reported when administered concurrently with NSAIDs

Follow-up

Further Inpatient Care

• Hospital admission for temporal arteritis is unusual but may be indicated depending upon the severity of symptoms and the ability of the patient to provide self-care at home.

Further Outpatient Care

• Most patients can be treated on an outpatient basis.
• An adequate quantity of corticosteroids should be prescribed.
• Follow-up care should be arranged within 72 hours.
• Symptoms typically improve within 1-3 days.
• Corticosteroid therapy may last for 1-2 years, depending on the patient’s response.

Inpatient & Outpatient Medications

• Nonsteroidal anti-inflammatory drugs can provide pain relief.
• Methotrexate⁵ and azathioprine have been used as both adjuncts and steroid-sparing agents for temporal arteritis, but conclusive evidence regarding their efficacy remains uncertain. These medications should not be prescribed from the emergency department, but they may be added at a later time upon rheumatology follow-up.
• Long-term steroid use (greater than 3 wk) may require the addition of calcium, vitamin D, and bisphosphonate therapy to prevent steroid-induced osteoporosis.

Transfer

• Hospital transfer is indicated only if visual disturbance is severe and cannot be adequately evaluated and managed at the current facility.

Complications

• Permanent vision loss is the most feared complication of untreated temporal arteritis and can even progress in some cases despite the initiation of corticosteroid therapy. This will tend to occur within the first 5 days of treatment if therapy is going to fail. As an outpatient, corticosteroid doses should be increased until symptoms improve.
• Approximately 50% of patients with temporal arteritis experience at least one flare-up that requires prolonged corticosteroid therapy.
• Patients with temporal arteritis are at increased risk for thoracic and abdominal aortic aneurysms compared to age-matched controls.
• Uncommon complications include CVA, memory loss, myocardial infarction, and peripheral neuropathy.

Prognosis

• Generally, temporal arteritis is a self-limiting condition lasting up to 2 years.
• Treatment with corticosteroids has proven to be effective in most cases, but the lengthy duration of treatment can lead to corticosteroid-induced complications.

Patient Education

• Timely follow-up care from the emergency department is critical to accurately diagnose temporal arteritis.
• Medication compliance and instructions to return to the emergency department if the condition worsens should be emphasized.
• Patients should be counseled that existing visual loss prior to arrival at the emergency department may not be regained despite initiation of therapy.

Miscellaneous

Medicolegal Pitfalls

• Failure to consider the diagnosis in elderly patients, especially with an elevated ESR
• Failure to consider other causes of systemic illness with an elevated ESR, such as malignancy or chronic infections
• Failure to promptly initiate corticosteroid therapy
• Failure to administer corticosteroids pending results of temporal artery biopsy
• Failure to arrange timely follow-up care

Special Concerns

• Emergency physicians should suspect temporal arteritis as a possible diagnosis in any older patient who presents with a new headache, jaw claudication, or symptoms of polymyalgia rheumatica, particularly those with an elevated ESR, CRP, or thrombocytosis.

Multimedia

Media file 1: Hematoxylin and eosin stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of giant cell arteritis shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.

Media file 2: Lumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis in the same patient whose temporal artery biopsy specimen is shown in Media file 1.

Media file 3: Hematoxylin and eosin stained femoral artery branch, cross section, taken from a lower limb amputation specimen from the same patient shown in Media files 1-2. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from giant cell arteritis resulted in ischemic necrosis of the lower limb, necessitating amputation.

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Keywords

TA, temporal arteritis, GCA, giant cell arteritis, cranial arteritis, vasculitis, systemic vasculopathy, temporal-located headaches, ischemic optic neuritis, headache, cephalgia, impaired vision, vision loss, jaw claudication, sixth nerve palsy, afferent pupillary defect, temporalarteritis, ophthalmic emergency, blindness, arteritic ischemic optic neuropathy 

Contributor Information and Disclosures

Author

Christopher H Lee, MD, Clinical Instructor, Section of EMS, Department of Emergency Medicine, Yale University School of Medicine
Christopher H Lee, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

Jean Marie Hammel, MD, Assistant Professor, Associate Residency Director of Emergency Medicine Residency Program, Department of Surgery, Section of Emergency Medicine, Yale University School of Medicine
Jean Marie Hammel, MD is a member of the following medical societies: Alpha Omega Alpha and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Richard S Krause, MD, Senior Faculty, Department of Emergency Medicine, State University of New York at Buffalo School of Medicine
Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gino A Farina, MD, Program Director, Associate Professor of Clinical Emergency Medicine, Department of Emergency Medicine, Long Island Jewish Medical Center, Albert Einstein College of Medicine
Gino A Farina, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Rick Kulkarni, MD, Medical Director, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
Rick Kulkarni, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: WebMD Salary Employment

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Ann G Egland, MD, and Leslie W Jackson, MD, to the development and writing of this article.

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