eMedicine Specialties > Dermatology > Diseases of the Vessels

Temporal (Giant Cell) Arteritis

John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
Victor J Marks, MD, Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System; Hyland Cronin, MD, Resident, Dermatology Department, Geisinger Health System, Danville, Pennsylvania

Updated: May 29, 2009

Introduction

Background

Temporal arteritis (TA), also known as cranial arteritis or giant cell arteritis, was first clinically recognized in 1890 when Hutchinson described an 80-year-old man whose painful inflamed temporal arteries precluded his wearing a hat. In 1932, Horton et al correlated the histopathologic features with the clinical features and applied the name arteritis temporalis. Other names include arteritis cranialis, Horton disease, granulomatous arteritis, and arteritis of the aged.

Since Horton's first description of temporal arteritis in the United States, this form of systemic vasculitis has become more widely recognized, as has the potential for serious sequelae, such as blindness and death. In 1960, Paulley and Hughes described the broad clinical spectrum and variable manifestations of the disease. Temporal arteritis is the most common form of systemic vasculitis in adults. Temporal arteritis primarily affects medium-sized arteries of the head and the neck. Most signs and symptoms reflect this distribution and include visual disturbances, headache, jaw claudication, neck pain, and scalp tenderness.

Although varied histopathologic findings have been noted over the years, temporal artery biopsy (TABx) remains the criterion standard for diagnosis of this granulomatous vasculitis. Until discovery of the anti-inflammatory properties of corticosteroids, patients with temporal arteritis received only supportive symptomatic treatment. Since 1950, steroids have revolutionized the treatment of the disease. Treatment with systemic corticosteroids produces a predictable clinical response and may prevent permanent visual loss, the most feared ischemic complication of the vasculitis.

Pathophysiology

Studies by O'Brien and Regan¹ have focused on the pathophysiologic role of actinic damage in persons with temporal arteritis. Chronic solar damage to elastic fibers may initiate an elastase-mediated degradation of the internal elastic lamina in temporal arteries, which may evoke an autoimmune response, resulting in granulomatous vasculitis.

The model of pathogenesis developed by Weyand and Goronzy² proposes that an as yet undetermined antigen is first encountered in the adventitia. T cells localized to this layer are activated and produce interferon gamma, which stimulates macrophage differentiation and migration. Giant cells are formed and secrete growth factors and metalloproteinases that ultimately degrade the internal elastic lamina and produce occlusive luminal hyperplasia. Potential antigens include elastin, viral or bacterial epitopes, and other extracellular matrix proteins.

Finally, numerous studies now suggest that temporal arteritis consists of various clinical subsets rather than one uniform disease. Variable expression of different cytokine profiles likely determines the clinical manifestations. Tumor necrosis factor (TNF) and, more recently, interleukin 6, have been recognized to may play a major role in the pathophysiology of temporal arteritis.³

Frequency

United States

Temporal arteritis is one of the most prevalent of the systemic vasculitis syndromes, with an estimated prevalence of 200 cases per 100,000 people older than 50 years and an incidence of 20-30 new cases per 100,000 persons older than 50 years. Although the condition had been considered less common among Hispanic persons, recent evidence has challenged this notion.

International

The incidence, age, and sex distribution in northern Europe is similar to that in the United States, likely because of immigration patterns. Latitude is an important risk factor, with a prevalence in Sweden twice that of Spain or Italy.

Mortality/Morbidity

Mortality from temporal arteritis is extremely uncommon, especially with timely diagnosis and treatment. In 1972, Wilkinson and Russell⁴ reviewed 12 autopsy cases and reported a high prevalence of severe involvement of the superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries and less frequent severe disease of the internal carotid, external carotid, and central retinal arteries. Intracranial arteries were never involved.

These patients, who died during the active phase of temporal arteritis, demonstrated a high prevalence of monocular blindness, occipital blindness, and brainstem strokes. Insignificant vasculitis was rarely identified in aortic, suprarenal, and coronary arteries and did not contribute to death. A close correlation existed between the amount of elastic tissue in susceptible arteries and the severity of the vasculitis. Subsequent morbidity and mortality from temporal arteritis reflect this anatomical distribution of vessels.

An insidious onset of the disease usually progresses to include fatigue, malaise, fever, weight loss, polymyalgia rheumatica, and flulike symptoms. Headache, scalp tenderness, focal arterial tenderness, jaw claudication, and visual loss may develop, and these findings indicate vascular involvement.

Race

Temporal arteritis primarily affects whites, specifically those of northern European descent. Scandinavians have the highest prevalence, and temporal arteritis is rare in African Americans. The gene for HLA-DRB1-04 has been identified as a risk factor for temporal arteritis. Although the condition had been considered less common among Hispanic persons, recent evidence has challenged this notion.

Sex

Females are affected in 80% of cases. Smoking increases the temporal arteritis risk 6-fold in women, while diabetes reduces the risk by half.

Age

Temporal arteritis is extremely uncommon in younger age groups. The average age at onset is in the sixth to seventh decade of life, with a mean age at onset of 69 years. It affects the same older population as the commonly associated polymyalgia rheumatica.

Clinical

History

• Temporal arteritis often manifests in an insidious manner with vague constitutional symptoms such as malaise, weight loss, fever, and fatigue.
• The classic manifestations are fever, anemia, headache, and an elevated erythrocyte sedimentation rate (ESR).
• Polymyalgia rheumatica (PMR) may develop either before or after the arteritis.
• Disease onset may also be abrupt. The diagnosis should be strongly suspected in whites older than 50 years of northern European descent, especially women, who report temporal headache, tender scalp, jaw claudication, and/or visual changes.
• Visual disturbances include diplopia, blurred vision, amaurosis fugax, and blindness in either one eye or both eyes.
• Symptoms signaling peripheral neuropathy or aortic involvement are rare.
• A history of chronic sun exposure may be relevant.
• In 2003, Amor-Dorado et al⁵ reported a previously unrecognized high incidence of audiovestibular disturbances such as vestibular dysfunction and/or hearing impairment in their temporal arteritis patients. Another rare complication involved lower extremity involvement with rapidly progressive claudication signaling compromised vascular flow. Surgery was often required.

Physical

• The examination should include an assessment of vital signs and blood pressure in both arms to rule out aortic arch involvement.
  • The scalp may be focally or generally tender. Temporal arteries may be tender, inflamed, dilated, thickened, or cordlike.
  • The arteries may be pulsatile, particularly early in the disease course.
  • Bruits may signal partial occlusion.
  • In severe cases, scalp skin or oral mucosal ulceration develops.
• Neurologic examination may reveal a neuropathy.
• Ophthalmologic examination reveals ischemic optic neuropathy in early stages of the disease and optic atrophy later in the disease course. Immediate referral is critical to ensure prompt diagnosis of reversible retinal ischemia from ophthalmic or posterior ciliary artery involvement.
• Audiovestibular examination may be considered.

Causes

The exact etiology of temporal arteritis remains unknown. See Pathophysiology.

Differential Diagnoses

Amyloidosis, Primary Systemic
Wegener Granulomatosis

Other Problems to Be Considered

PMR is a closely correlated process with similar systemic features but without the focal symptoms and signs caused by temporal or ophthalmic artery disease.

Wegener granulomatosis and polyarteritis nodosa may occasionally affect the temporal artery and cause clinical findings similar to temporal arteritis (TA). Other vasculitides, such as lupus erythematosus, rheumatoid arthritis, and Takayasu arteritis, may have similar signs and symptoms.

Systemic amyloidosis rarely affects temporal arteries and may cause jaw or arm claudication.

Workup

Laboratory Studies

• At the time of diagnosis, the ESR typically ranges from 60-100 mm/h. Rarely, it may be normal. A highly elevated ESR is more characteristic for temporal arteritis than for other vasculitides and rheumatologic diseases. The ESR may be followed serially to help monitor response to therapy and to titrate steroid dosing.
• Other acute-phase reactant levels are elevated and reflect the underlying inflammatory process. Some evidence indicates that interleukin 6 may be more sensitive than the ESR and C-reactive protein level as disease-related acute-phase reactants and, therefore, may be a better predictor of disease flare; however, currently this test is not readily available in most laboratories.³ Hepatic enzyme levels, particularly alkaline phosphatase, are elevated in 20-30% of cases.
• Five studies have documented that thrombocytosis (platelet count >375,000/µL) is more helpful for ruling in temporal arteritis than an elevated ESR. Conversely, a normal platelet count is more accurate for ruling out temporal arteritis than a normal ESR.⁶

Imaging Studies

• Serious suspicion for aortic or carotid artery disease may rarely warrant noninvasive studies (eg, CT scanning) or invasive angiography. Angiography is less sensitive, less specific, and more invasive than biopsy. See Temporal Arteritis for additional discussion.
• Color-duplex ultrasonography has recently been suggested as an alternative to temporal artery biopsy (TABx) for diagnosing temporal arteritis. Schmidt et al⁷ observed a halo effect surrounding actively involved temporal arteries that was absent in healthy controls. They suggested that TABx might be replaced by ultrasonography when classic symptoms are present. Salvarani et al⁸ confirmed the high specificity of the halo effect, which results from adventitial edema, but reported a sensitivity of only 40%. In particular, early inflammatory changes diagnosed based on TABx findings do not produce the characteristic halo effect. Therefore, ultrasonography should not replace TABx for diagnosing temporal arteritis in clinical practice. Two recent studies are still challenging this theory.^9,10
• Positron-emission tomography, MRI, and CT scanning also lack the sensitivity of TABx in the diagnosis of temporal arteritis. These noninvasive techniques, however, may play a future role in the monitoring and long-term management of temporal arteritis.^9,10

Procedures

• TABx is the criterion standard for diagnosing temporal arteritis. This procedure may be performed by ophthalmologists, general surgeons, head and neck surgeons, plastic or vascular surgeons, or dermatologic surgeons. With a positive predictive value of greater than 90%, TABx stands out as the definitive test for temporal arteritis. It has helped to determine appropriate treatment in 94% of suspected cases of temporal arteritis.
  • The frontal branch of the superficial temporal artery is preoperatively identified by using Doppler ultrasonography and then marked with a pen or with dye. In approximately 16% of cases, the frontal branch is atrophic or absent, in which case a biopsy should be performed on the main trunk of the superficial temporal artery using a preauricular approach.¹¹ With the patient under local anesthesia, a shallow incision just into the underlying fat is made directly over the artery. The artery is bluntly dissected free from within the superficial temporal fascia. A segment of artery is ligated proximally and distally, removed, and sent for histopathologic review. Hemostasis is obtained with electrocoagulation, and a layered closure is performed.
  • To improve the yield and to avoid complications, proper site selection is important. Focal symptoms or signs, such as erythema, tenderness, absent pulsations, arterial nodularity or swelling, inflammation, bruit, or thickening, help guide biopsy site selection and may improve the yield of the biopsy. However, localizing findings are often absent or misleading, and, frequently, a poor correlation is noted between the physical examination findings and the biopsy results. In the absence of localizing findings, the danger zone of the temporal branch of the facial nerve is avoided. Knowledge of the anatomy and careful dissection above and within the superficial temporal fascia help prevent nerve damage during the procedure. 
  • Of particular importance, the zygomatic arch is the most risky location for injury to the temporal branch of the facial nerve. Injury to this nerve leads to an inability to elevate the eyebrows, brow ptosis, paralysis, and a possible asymmetric appearance of the forehead due to loss of the lines and wrinkles on the affected side. A line drawn from the earlobe to the lateral edge of the eyebrow, and from the tragus to just above and behind the highest forehead crease (or 2 cm above supraorbital rim) helps to outline the danger zone where the nerve is superficial. Importantly, undermine just below the dermis in the superficial fat and above the superficial fascia.¹²
  • Most authors recommend excision of 2-5 cm of artery to provide accurate diagnosis of temporal arteritis because studies have noted higher positive rates with longer specimens. Temporal arteritis may have a patchy distribution among extracranial arteries and within small segments of these arteries. Longer biopsy specimens provide more tissue in which to demonstrate short, noncontiguous foci of giant cell arteritis, the so-called skip areas. These skip areas are not commonly identified, but patches of arteritis as short as 0.29 mm have been clearly demonstrated on serial sectioning. Serial sectioning, proper tissue handling, and adequate specimen length are critical to ensure maximum yield from the biopsy.
  • Because of the segmental nature of temporal arteritis, a subsequent TABx from the opposite side of a negative biopsy sample occasionally shows evidence of disease. Therefore, some authors recommend initial bilateral biopsy, while others repeat the procedure only in the setting of further clinical suspicion. Four retrospective reviews have reported that only 1-5% of true positives would be confirmed with a bilateral biopsy in the setting of a first negative biopsy result.
  • Additionally, a retrospective study showed that 90 of 91 patients who underwent bilateral TABx showed the same histology, with a concordance rate of 98.9% among positive results. The one discordant pair involved arterial samples that were very short (4 mm and 6 mm), thus further emphasizing the need for longer sections.¹³
  • Another study based on a Bayesian statistical technique reported the sensitivity of a single TABx to be 87.1%¹⁴   
  • Even with these results, however, most physicians with high clinical suspicion despite initial negative biopsy findings would still recommend a second contralateral biopsy, given the consequences of a missed diagnosis.
  • TABx is a safe procedure; however, risks include temporary or permanent damage to the temporal branch of the facial nerve, infection, bleeding, hematoma, and dehiscence. Isolated case reports of ischemic stroke or skin ulceration from disruption of collateral flow from an asymptomatic carotid occlusion can be found.

Histologic Findings

Involved vessels show a panarteritis that may be patchy and segmental in its distribution. Degeneration of the internal elastic lamina invokes an inflammatory response. The composition of the inflammatory infiltrate changes in a time-dependent manner. Some neutrophils may be noted in early lesions; later, mononuclear cells and giant cells predominate. Macrophages digest the elastic material and evolve into multinucleated giant cells. Lymphocytes infiltrate the vessel at all levels. Despite the inflammation, the arterial wall does not become necrotic. Intimal deposition of fibrinous material causes luminal narrowing and eventual obliteration.

Although giant cells are characteristic, they may rarely be absent. In one study, those with giant cells had nearly 3 times the occurrence of blindness and PMR compared with those without giant cells. Although not statistically significant, with more patients this finding may reach statistical significance.¹⁵

Treatment

Medical Care

• Medical care for temporal arteritis (TA) is supportive and symptom specific.

Consultations

• Consultation with an ophthalmologist is critical for all patients suspected of having temporal arteritis in order to prevent any permanent visual deficit.
• Rheumatologists often treat these patients, although referral is not essential.

Medication

Since 1950, the use of systemic corticosteroids has revolutionized the treatment of temporal arteritis, as well as a legion of other inflammatory diseases. Supportive care was the primary treatment before steroid therapy. Now, patients with temporal arteritis respond quickly and often completely to systemic steroids.

Despite corticosteroids serving as the mainstay of therapy, no consensus exists regarding a standard initial dose, maintenance dosing, or alternate-day schedules. Authorities do agree that the initiation of steroid therapy should not be delayed because TABx can still confirm the diagnosis and serious sequelae can be avoided.

An initial dose of 40-60 mg/d of prednisone (or equivalent) as a single or divided dose is generally found to be adequate in the vast majority of the cases. It is usually given for 2-4 weeks until all reversible signs and symptoms have resolved and levels of acute-phase reactants are back to normal. The dose is then gradually reduced every 1-2 weeks by a maximum of 10% of the total daily dose. Most patients are treated for 1-2 years, but some with a prolonged or relapsing course may require low doses of glucocorticoids (GCs) for several years. Clinical flares usually occur when the prednisone is reduced to 5-10 mg/d.

Higher-dose pulse GC therapy has been advocated by some for patients with recent or pending visual disturbances, but an observational study and a randomized controlled trial failed to demonstrate superiority of pulse therapy over oral GC therapy in preventing ischemic complications. The benefit conferred by GCs needs to be balanced against the common and well-recognized complications related to long-term GC use. In a population-based study of 120 patients with temporal arteritis, 86% of patients had adverse events, including bone fractures, avascular necrosis of the hip, diabetes mellitus, infections, GI bleeding, and cataracts. Adverse events were related to the age of patients and a high cumulative dose of GCs. Therefore, randomized controlled trials need to be performed to define the minimal effective starting GC dosage.

Furthermore, much of the total morbidity of temporal arteritis is not related to the disease itself, but to the impact of long-term GC therapy in a population of elderly patients. Therefore, most treatment studies in which other drugs have been added to GCs have been aimed at early reduction of steroids while maintaining the suppression of temporal arteritis for the duration of time the disease evolves through its natural course to become inactive. Only methotrexate (MTX), azathioprine, and the TNF-blockers infliximab and etanercept have been tested in randomized controlled trials.

A formal meta-analysis has suggested that adjunctive MTX treatment in dosages of 7.5-15 mg/wk for temporal arteritis reduced the risk of a first relapse by 35% and of a second relapse by 51%. In addition, MTX reduced the cumulative exposure to GCs. However, the superiority of the treatment effect of MTX over placebo fully appeared only after a latency period of 24-36 weeks, and no between-group difference was noted in the occurrence of adverse events.

The benefit observed in the azathioprine-treated patients appeared to be unimpressive and of late onset.

Another randomized controlled trial showed that adding infliximab to GCs provided no measurable benefit in the management of newly diagnosed temporal arteritis.

Finally, a double-blind, placebo-controlled trial was performed to test the hypothesis that TNF inhibition with etanercept could reduce GC exposure in patients with refractory temporal arteritis requiring a stable dose of prednisone of 10 mg/d for maintaining clinical remission and with at least one GC-related adverse effect. The results showed that patients in the etanercept group were more successful in discontinuing prednisone therapy and they required a significantly lower cumulative prednisone dose. By contrast, no difference was noted in the number and type of adverse events. Thus, at least for the time being, anti-TNF therapy can be considered in patients with longstanding GC-resistant temporal arteritis who are at risk of GC-related adverse events. A larger trial with longer follow-up is needed to determine if TNF blockers are able to reduce the cumulative GC dosage and decrease GC-associated morbidity. Until more data are available, GCs remain the drug of choice.^3,16

Other possible therapeutic drug options include cyclophosphamide, cyclosporine, dapsone, toclizumab (humanized monoclonal anti-interleukin 6 receptor antibody), rituximab (anti-CD20 monoclonal antibody), and abatacept (recombinant fusion protein that modulates CD28-mediated T cell co-stimulation).³ Generally, none is routinely recommended.

Several clinical trials are either actively recruiting or are active but not yet recruiting. For a complete list, see these List Results from ClinicalTrials.gov.

Corticosteroids

Anti-inflammatory action is effective in the treatment of granulomatous vasculitis.

Prednisone (Deltasone, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN leukocyte activity.
Oral therapy is standard choice for outpatient management, but intravenous and intramuscular alternatives may be options for unique situations.

Dosing

Adult

1 mg/kg/d PO or 80-100 mg/d PO for first mo; as clinical and laboratory parameters improve, taper and titrate dose

Pediatric

4-5 mg/m²/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve

Interactions

Ketoconazole, erythromycin, clarithromycin, estrogens, and birth control pills increase levels
Aminoglutethimide, phenytoin, phenobarbital, rifampin, cholestyramine, and ephedrine decrease steroid levels
Potassium-depleting diuretics (potentiate potassium loss and digitalis toxicity) and cyclosporine increase levels
Isoniazid, insulin (resistance is induced), and salicylates decrease levels
Monitor anticoagulant therapy and theophylline levels

Contraindications

Absolute: Systemic fungal infection; herpes simplex keratitis; hypersensitivity.
Relative: Hypertension, active tuberculosis, congestive heart failure, prior psychosis, positive PPD test, glaucoma, severe depression, diabetes, active peptic ulcer disease, cataracts, osteoporosis, recent bowel anastomosis, pregnancy.

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

Abrupt discontinuation of GCs may cause adrenal crisis; because of vision complications and potentially life-threatening nature of temporal arteritis, steroid treatment is rarely withheld; well-known adverse effects of systemic steroids are managed prophylactically and systematically depending on the patient
Caution in hypertension, CHF, severe depression or prior psychosis, active peptic ulcer disease, recent bowel anastomosis, active tuberculosis, positive PPD test, diabetes mellitus, osteoporosis, glaucoma, cataracts, and pregnancy; elderly patients are particularly susceptible to many adverse effects, including hyperglycemia, fluid retention, hypertension, hyperlipidemia, benign intracranial pressure, hypokalemia, enhanced neuromuscular blockade, GI bleeding, myopathy, psychosis, osteoporosis, and drug interactions
Patients requiring long-term treatment benefit from increased exercise and calcium and vitamin D supplementation; qod dosing; diets low in calories, fat, and sodium and high in protein, potassium, and calcium; avoid alcohol, coffee, and nicotine; hormonal supplementation for menopausal women

Prednisolone (AK-Pred, Delta-Cortef, Articulose-50, Econopred)

Decreases inflammation by suppressing migration of PMN leukocytes and reducing capillary permeability.

Dosing

Adult

5-60 mg/d PO/IV/IM

Pediatric

0.1-2 mg/kg PO/IV/IM qd or divided tid/qid

Interactions

Decreases effects of salicylates and toxoids (for immunizations); phenytoin, carbamazepine, barbiturates, and rifampin decrease effects

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular skin infections

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

Caution in hyperthyroidism, osteoporosis, cirrhosis, nonspecific ulcerative colitis, peptic ulcer, diabetes, and myasthenia gravis

Methylprednisolone (Solu-Medrol, Depo-Medrol)

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

Dosing

Adult

2-60 mg PO qd or divided bid/qid followed by gradual reduction to lowest level that maintains clinical response

Pediatric

0.5-1.7 mg/kg/d or 5-25 mg/m²/d PO/IV/IM divided q6-12h

Interactions

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

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular skin infections

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 GC use

Follow-up

Deterrence/Prevention

• Sun avoidance and protection of the head and the face from photodamage may eventually prove to be important preventive measures for temporal arteritis (TA).

Complications

• Ophthalmic complications are common.
  • Clinical manifestations include visual disturbances, amaurosis fugax, diplopia, and blindness.
  • Visual loss may be temporary or permanent and partial or complete in either one eye or both eyes.
  • Blindness usually occurs abruptly and painlessly.
  • Ischemic optic neuritis with eventual optic atrophy is the most common cause of visual loss and occurs in 15% of patients.
  • Mesenteric vasculitis resulting in small bowel infarction has rarely been described in persons with temporal arteritis, but it represents a serious and potentially treatable complication.¹⁷

Prognosis

• Overall, with prompt diagnosis and treatment, temporal arteritis is a well-controlled disease. Symptoms from temporal arteritis improve within days of treatment. Corticosteroids can usually be tapered within the first 4-6 weeks and eventually discontinued. The prognosis for patients is excellent.
  • Some patients' conditions respond well, but damage prior to treatment is irreversible.
  • Rarely, a response to steroid therapy does not occur or doses cannot be tapered. Cytotoxic or immunosuppressive drugs have been recommended, but more data are needed.

Miscellaneous

Medicolegal Pitfalls

• Improper technique during TABx can rarely result in facial nerve damage. Understanding the temporal anatomic danger zone and planning the procedure accordingly, with execution within the superficial temporal fascia, can prevent nerve injury.

References

1. O'Brien JP, Regan W. Actinically degenerate elastic tissue is the likely antigenic basis of actinic granuloma of the skin and of temporal arteritis. J Am Acad Dermatol. Feb 1999;40(2 Pt 1):214-22. [Medline].

2. Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. Jul 10 2003;349(2):160-9. [Medline].

3. Salvarani C, Pipitone N, Boiardi L, Hunder GG. Do we need treatment with tumour necrosis factor blockers for giant cell arteritis?. Ann Rheum Dis. May 2008;67(5):577-9. [Medline].

4. Wilkinson IM, Russell RW. Arteries of the head and neck in giant cell arteritis. A pathological study to show the pattern of arterial involvement. Arch Neurol. Nov 1972;27(5):378-91. [Medline].

5. Amor-Dorado JC, Llorca J, Garcia-Porrua C, Costa C, Perez-Fernandez N, Gonzalez-Gay MA. Audiovestibular manifestations in giant cell arteritis: a prospective study. Medicine (Baltimore). Jan 2003;82(1):13-26. [Medline].

6. Ellen H, Nusser J. Which clinical features and lab findings increase the likelihood of temporal arteritis?. The Journal of Family Practice. 2008;57:119-120.

7. Schmidt WA, Kraft HE, Vorpahl K, Völker L, Gromnica-Ihle EJ. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med. Nov 6 1997;337(19):1336-42. [Medline].

8. Salvarani C, Silingardi M, Ghirarduzzi A, et al. Is duplex ultrasonography useful for the diagnosis of giant-cell arteritis?. Ann Intern Med. Aug 20 2002;137(4):232-8. [Medline].

9. Alberts M, Mosen D. Diagnosing temporal arteritis: duplex vs biopsy. Quarterly Journal of Medicine. 2007;100:785-789.

10. Bley TA, Reinhard M, Hauenstein C, Markl M, Warnatz K, Hetzel A. Comparison of duplex sonography and high-resolution magnetic resonance imaging in the diagnosis of giant cell (temporal) arteritis. Arthritis Rheum. Aug 2008;58(8):2574-8. [Medline].

11. Marano SR, Fischer DW, Gaines C, Sonntag VK. Anatomical study of the superficial temporal artery. Neurosurgery. Jun 1985;16(6):786-90. [Medline].

12. Wolff K et al. Fig 243-4. In: Fitzpatrick's Dermatology In General Medicine. 7th. 2008:2291.

13. DAnesh-Meyer H, Eagle R, Kubis K, Savineo P, and Sergott R. Low Diagnositc Yield with Second Biopsies in Suspected Giant Cell Arteritis. Journal of Neuro-Opthalmology. 2000;20:213-215.

14. Levin L, Nuiederkohr R. A Bayesian Analysis of the True Sensitivity of a Temporal Artery Biopsy. Investigative Opthalmology & Visual Science. 2007;48:675-680.

15. Armstrong AT, Tyler WB, Wood GC, Harrington TM. Clinical importance of the presence of giant cells in temporal arteritis. J Clin Pathol. May 2008;61(5):669-71. [Medline].

16. Martinez-Taboada VM, Rodriguez-Valverde V, Carreno L, Lopez-Longo J, Figueroa M, Belzunegui J. A double-blind placebo controlled trial of etanercept in patients with giant cell arteritis and corticosteroid side effects. Ann Rheum Dis. May 2008;67(5):625-30. [Medline].

17. Cuizhen K, Scula C. Mesenteric Involvement in Giant Cell Arteritis. An Underrecognized Complication?: Analysis of a Case Series with Clinicoanatomic Correlation. Medicine. 2008;87:45-51.

18. Albert DM, Searl SS, Craft JL. Histologic and ultrastructural characteristics of temporal arteritis. The value of the temporal artery biopsy. Ophthalmology. Oct 1982;89(10):1111-26. [Medline].

19. Albertini JG, Ramsey ML, Marks VJ. Temporal artery biopsy in a dermatologic surgery practice. Dermatol Surg. Jun 1999;25(6):501-8. [Medline].

20. Bevan AT, Dunnill MS, Harrison MJ. Clinical and biopsy findings in temporal arteritis. Ann Rheum Dis. May 1968;27(3):271-7. [Medline].

21. Birkhead NC, Wagener HP, Shick RM. Treatment of temporal arteritis with adrenal corticosteroids; results in fifty-five cases in which lesion was proved at biopsy. J Am Med Assoc. Mar 9 1957;163(10):821-7. [Medline].

22. Brennan J, McCrary JA 3rd. Diagnosis of superficial temporal arteritis. Ann Ophthalmol. Aug 1975;7(8):1125-9. [Medline].

23. Chmelewski WL, McKnight KM, Agudelo CA, Wise CM. Presenting features and outcomes in patients undergoing temporal artery biopsy. A review of 98 patients. Arch Intern Med. Aug 1992;152(8):1690-5. [Medline].

24. Fauchald P, Rygvold O, Oystese B. Temporal arteritis and polymyalgia rheumatica. Clinical and biopsy findings. Ann Intern Med. Dec 1972;77(6):845-52. [Medline].

25. Fernandez-Herlihy L. Temporal arteritis: clinical aids to diagnosis. J Rheumatol. Dec 1988;15(12):1797-801. [Medline].

26. Hall S, Persellin S, Lie JT, O'Brien PC, Kurland LT, Hunder GG. The therapeutic impact of temporal artery biopsy. Lancet. Nov 26 1983;2(8361):1217-20. [Medline].

27. Hedges TR 3rd, Gieger GL, Albert DM. The clinical value of negative temporal artery biopsy specimens. Arch Ophthalmol. Aug 1983;101(8):1251-4. [Medline].

28. Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR. Temporal arteritis: a 25-year epidemiologic, clinical, and pathologic study. Ann Intern Med. Feb 1978;88(2):162-7. [Medline].

29. Kelley JS. Doppler ultrasound flow detector used in temporal artery biopsy. Arch Ophthalmol. May 1978;96(5):845-6. [Medline].

30. Kent RB 3rd, Thomas L. Temporal artery biopsy. Am Surg. Jan 1990;56(1):16-21. [Medline].

31. Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc. Aug 1976;51(8):504-10. [Medline].

32. Lam BL, Wirthlin RS, Gonzalez A, Dubovy SR, Feuer WJ. Giant cell arteritis among Hispanic Americans. Am J Ophthalmol. Jan 2007;143(1):161-3. [Medline].

33. Levine SM, Hellmann DB. Giant cell arteritis. Curr Opin Rheumatol. Jan 2002;14(1):3-10. [Medline].

34. Nordborg E, Nordborg C. Giant cell arteritis: epidemiological clues to its pathogenesis and an update on its treatment. Rheumatology (Oxford). Mar 2003;42(3):413-21. [Medline].

35. Nordborg E, Nordborg C. Giant cell arteritis: strategies in diagnosis and treatment. Curr Opin Rheumatol. Jan 2004;16(1):25-30. [Medline].

36. Schumacher HR, Klippel JH, Koopman WJ, eds. Vasculitis. In: Primer on the Rheumatic Diseases. 10^th ed. Atlanta, Ga: Arthritis Foundation; 1993:136-48.

37. Seko Y. Giant cell and Takayasu arteritis. Curr Opin Rheumatol. Jan 2007;19(1):39-43. [Medline].

38. Sorensen S, Lorenzen I. Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. A retrospective study of 63 patients. Acta Med Scand. 1977;201(3):207-13. [Medline].

39. Weyand CM, Bartley GB. Giant cell arteritis: new concepts in pathogenesis and implications for management. Am J Ophthalmol. Mar 1997;123(3):392-5. [Medline].

Keywords

temporal arteritis, giant cell arteritis, arteritis temporalis, arteritis cranialis, Horton disease, Horton's disease, granulomatous arteritis, arteritis of the aged, cranial arteritis, systemic vasculitis, TA, giant cell arteritis, temporal arteritis

Contributor Information and Disclosures

Author

John G Albertini, MD, Consulting Staff, Dermatologic Surgery, The Skin Surgery Center
John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology and American College of Mohs Micrographic Surgery and Cutaneous Oncology
Disclosure: Nothing to disclose.

Coauthor(s)

Victor J Marks, MD, Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System
Victor J Marks, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physicians, American Medical Association, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Hyland Cronin, MD, Resident, Dermatology Department, Geisinger Health System, Danville, Pennsylvania
Disclosure: Nothing to disclose.

Medical Editor

Russell Hall, MD, Chief, Professor, Department of Internal Medicine, Division of Dermatology, Duke University
Russell Hall, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, American Society for Clinical Investigation, and Society for Investigative Dermatology
Disclosure: Genetech Grant/research funds Principle Investigator; Centecor  Grant/research funds Principle Investigator

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)