Temporal/Giant Cell Arteritis Treatment & Management

  • Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP; Chief Editor: B Mark Keegan, MD, FRCPC   more...
 
Updated: Feb 3, 2012
 

Medical Care

Regardless of extent of neurologic involvement, oral corticosteroids remain the mainstay of treatment.

  • Steroid-related adverse effects
    • The frequent and potentially serious consequences of chronic steroid therapy (eg, diabetes mellitus, vertebral compression fractures, steroid myopathy, steroid psychosis, immunosuppression-related infections) have led many to question the use of chronic steroid therapy.
    • Some studies have suggested that much lower doses and more rapid tapering schedules than currently used are sufficient. One such study advocated initiating treatment with 20 mg of prednisolone daily and tapering it to 10 mg daily within 3 months.
    • Many patients may respond to this regimen with symptomatic improvement of headache, PMR, and reduction of ESR; however, a substantial number of patients experience worsening of symptoms.
    • Headache and PMR, although the most common symptoms of GCA, are not reasons for using high doses of steroids.
    • Higher doses of steroids are required to prevent irreversible ischemic ophthalmologic and neurologic complications, which may be an early manifestation of GCA or, less commonly, develop during a flare.
    • Despite the apparent higher incidence of ischemic eye and brain complications in patients with carotid bruits, a bruit is not a sufficient indicator to base a decision between a high- or low-dose steroid regimen.
  • Alternative immunosuppressant agents: Trials of other immunosuppressant agents, including azathioprine, methotrexate, dapsone, and cyclophosphamide, have been attempted for their steroid-sparing effects.
    • Steroid dosages have been lowered successfully but inconsistently in some patients on each of these drugs.
    • Toxicity can be a significant problem, particularly with dapsone and cyclophosphamide.
    • Azathioprine has no acute effect, and its steroid-sparing effects may not be evident for a year.
    • Limited experience suggests that cyclophosphamide may be the most consistently effective immunosuppressant. It may permit more rapid steroid tapering when instituted following a relapse.
    • Low-dose aspirin may be used as a prophylactic measure to prevent stroke because stroke may occur despite high-dose corticosteroid therapy and because almost all GCA patients have thrombocytosis.
  • In most cases, the neurologist should consult with a rheumatologist and the treating internist before instigating treatment with any of these alternative immunosuppressive agents.
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Consultations

GCA diagnosis and treatment involves neurologists, rheumatologists, ophthalmologists, neurosurgeons, and pathologists.

  • Rheumatology: GCA crosses the subspecialties of neurology and rheumatology, and neurologists uncomfortable with the diagnosis and management of GCA should consider rheumatologic consultation.
  • Ophthalmology: The funduscopic features of AION may require ophthalmologic consultation for diagnosis and treatment recommendations, particularly in subtle cases.
  • Neurosurgery
    • Surgical consultation is necessary for TAB.
    • Depending on institution, this procedure can be performed by a neurosurgeon, plastic surgeon, or general surgeon.
  • Pathology
    • That the treating neurologist be familiar and confident with the laboratory evaluating the TAB specimen is imperative, since this is the criterion standard for GCA diagnosis.
    • A myriad of laboratory errors (eg, specimen handling, fixation, sectioning) can, if they occur, result in a misdiagnosis (most often a false-negative result).
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Diet

Patients with GCA who are on steroid therapy should be monitored carefully for the steroid-related complications of diabetes mellitus, hypertension, peripheral edema, and weight gain.

  • Monitor dietary sugar, salt, and caloric intake to prevent such complications.
  • Patients on chronic steroid therapy are also at risk for gastric ulcer disease and should be placed on H2 blockers or antacids.
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Activity

No activity restrictions are necessary in a patient with GCA who is asymptomatic on adequate therapy.

  • If a patient has ischemic eye or brain symptoms, then bedrest in a supine position may be desirable before or when first beginning steroid therapy.
  • Some patients may have orthostatically sensitive amaurosis fugax, but this is rare.
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Contributor Information and Disclosures
Author

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

Coauthor(s)

Arun Ramachandran  State University of New York Upstate Medical University

Arun Ramachandran is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Jorge E Mendizabal, MD  Consulting Staff, Corpus Christi Neurology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed  Director, Regional MS Center of Excellence, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

B Mark Keegan, MD, FRCPC  Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting; Bionest Consulting fee Consulting

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Richard J. Caselli, MD to the development and writing of this article.

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Hematoxylin- and eosin-stained superficial temporal artery biopsy specimen, cross section. The hallmark histologic features of GCA shown here include intimal thickening with luminal stenosis, mononuclear inflammatory cell infiltrate with media invasion and necrosis, and giant cell formation in the media.
Lumbar angiogram showing stenosis and occlusion of femoral artery branches due to vasculitis.
Hematoxylin- and eosin-stained femoral artery branch, cross section, taken from a lower limb amputation specimen. Mononuclear cell invasion and necrosis in the media of this large artery can be observed. Extensive lower limb vasculitis from GCA resulted in ischemic necrosis of the lower limb, necessitating amputation.
 
 
 
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