eMedicine Specialties > Dermatology > Diseases of the Vessels
Temporal (Giant Cell) Arteritis: Treatment & Medication
Updated: May 29, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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.
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
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
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.
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.
Adult
5-60 mg/d PO/IV/IM
Pediatric
0.1-2 mg/kg PO/IV/IM qd or divided tid/qid
Decreases effects of salicylates and toxoids (for immunizations); phenytoin, carbamazepine, barbiturates, and rifampin decrease effects
Documented hypersensitivity; viral, fungal, or tubercular skin infections
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.
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
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
Documented hypersensitivity; viral, fungal, or tubercular skin infections
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
More on Temporal (Giant Cell) Arteritis |
| Overview: Temporal (Giant Cell) Arteritis |
| Differential Diagnoses & Workup: Temporal (Giant Cell) Arteritis |
Treatment & Medication: Temporal (Giant Cell) Arteritis |
| Follow-up: Temporal (Giant Cell) Arteritis |
| References |
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References
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].
Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. Jul 10 2003;349(2):160-9. [Medline].
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].
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].
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].
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.
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].
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].
Alberts M, Mosen D. Diagnosing temporal arteritis: duplex vs biopsy. Quarterly Journal of Medicine. 2007;100:785-789.
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].
Marano SR, Fischer DW, Gaines C, Sonntag VK. Anatomical study of the superficial temporal artery. Neurosurgery. Jun 1985;16(6):786-90. [Medline].
Wolff K et al. Fig 243-4. In: Fitzpatrick's Dermatology In General Medicine. 7th. 2008:2291.
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.
Levin L, Nuiederkohr R. A Bayesian Analysis of the True Sensitivity of a Temporal Artery Biopsy. Investigative Opthalmology & Visual Science. 2007;48:675-680.
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].
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].
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.
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].
Albertini JG, Ramsey ML, Marks VJ. Temporal artery biopsy in a dermatologic surgery practice. Dermatol Surg. Jun 1999;25(6):501-8. [Medline].
Bevan AT, Dunnill MS, Harrison MJ. Clinical and biopsy findings in temporal arteritis. Ann Rheum Dis. May 1968;27(3):271-7. [Medline].
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].
Brennan J, McCrary JA 3rd. Diagnosis of superficial temporal arteritis. Ann Ophthalmol. Aug 1975;7(8):1125-9. [Medline].
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].
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].
Fernandez-Herlihy L. Temporal arteritis: clinical aids to diagnosis. J Rheumatol. Dec 1988;15(12):1797-801. [Medline].
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].
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].
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].
Kelley JS. Doppler ultrasound flow detector used in temporal artery biopsy. Arch Ophthalmol. May 1978;96(5):845-6. [Medline].
Kent RB 3rd, Thomas L. Temporal artery biopsy. Am Surg. Jan 1990;56(1):16-21. [Medline].
Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc. Aug 1976;51(8):504-10. [Medline].
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].
Levine SM, Hellmann DB. Giant cell arteritis. Curr Opin Rheumatol. Jan 2002;14(1):3-10. [Medline].
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].
Nordborg E, Nordborg C. Giant cell arteritis: strategies in diagnosis and treatment. Curr Opin Rheumatol. Jan 2004;16(1):25-30. [Medline].
Schumacher HR, Klippel JH, Koopman WJ, eds. Vasculitis. In: Primer on the Rheumatic Diseases. 10th ed. Atlanta, Ga: Arthritis Foundation; 1993:136-48.
Seko Y. Giant cell and Takayasu arteritis. Curr Opin Rheumatol. Jan 2007;19(1):39-43. [Medline].
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].
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].
Further Reading
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
Treatment & Medication: Temporal (Giant Cell) Arteritis