eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Temporal/Giant Cell Arteritis: Treatment & Medication

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
Coauthor(s): Arun Ramachandran, State University of New York Upstate Medical University
Contributor Information and Disclosures

Updated: Jun 22, 2009

Treatment

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.

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).

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.

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.

Medication

Oral corticosteroids are the mainstay of treatment.

Glucocorticoids

These agents have anti-inflammatory properties. They cause profound and varied metabolic effects. In addition, they modify the body's immune response to diverse stimuli and inhibit the synthesis of TNF-alpha, IL-2, IL-6, and IFN-gamma. In addition, glucocorticoids modulate serum and leukocyte-bound levels of cell adhesion molecules.


Prednisone (Sterapred)

Commonly used oral corticosteroid. Must be metabolized in liver to active metabolite prednisolone. By suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability, decreases inflammation.
Typical patients require prednisone for 1-2 y with daily initial doses of 40-60 mg. In acute neurologic syndrome or rapidly worsening neurologic status—whether visual loss, mononeuritis multiplex, or acute encephalopathy—treatment may begin with IV pulses over several days.
A patient with GCA who has a relapse may require only a modest dose increment to control flare in symptoms. Following initiation of treatment, ESR may be expected to drop within days and become normal in 1-2 wk. All neurologic deficits can improve, but irreversible end-organ infarction may preclude clinically significant gains in some patients. Neurovascular complications may occur during initial tapering of corticosteroid dosage (often around 1 mo after beginning treatment), underscoring need for ESR monitoring and importance of small steroid decrements.
The doses described below are suggested for general consideration. Tailor dosing regimens to medical circumstances confronting patient.

Adult

No neurologic syndrome or stable neurologic status: 60 mg PO qd initially
Acute neurologic syndrome or rapidly worsening neurologic status: 120 mg PO qd; taper to approximately 40 mg PO qd by end of first mo; may reduce dose by 2.5-5 mg q2-3wk as tolerated

Pediatric

Not established

Clearance may decrease when used with estrogens; may increase digitalis toxicity secondary to hypokalemia in patients taking digoxin; metabolism may be increased by phenobarbital, phenytoin, or rifampin—consider increasing maintenance dose; monitor for hypokalemia when taken with diuretics

Documented hypersensitivity, viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, fungal or tubercular skin infections, GI disease

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

Long-term use may predispose patients to hyperglycemia, manifestation of latent diabetes mellitus, nonketotic hyperosmolar state, osteoporosis, avascular necrosis of hip, cataracts, steroid myopathy, cushingoid appearance, weight gain, suppression of pituitary-hypothalamic axis, peptic ulcer disease, suppression of growth (children), unmasking of latent infections (eg, tuberculosis, herpes zoster), increased predisposition to fungal and parasitic infections
Water retention resulting from therapy may precipitate congestive heart failure (CHF), hypertension, hypokalemia; suppression of pituitary-hypothalamic axis may cause patients to require higher doses at times of stress (eg, systemic infections, surgery)


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

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability. Use in acute neurologic reactions.

Adult

Acute neurologic syndrome or rapidly worsening neurologic status: 1000 mg IV qd; follow with prednisone PO dosing regimen when stable

Pediatric

Not established

May increase digitalis toxicity secondary to hypokalemia in patients taking digoxin; levels may be increased by estrogens; levels may be decreased by phenobarbital, phenytoin, and rifampin—may need to adjust dose; monitor patients for hypokalemia when taking 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

Possible adverse effects include hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections

Immunosuppressant agents

Inhibit key factors of the immune system.


Azathioprine (Imuran)

Inhibits mitosis and cellular metabolism by antagonizing purine metabolism and inhibiting synthesis of DNA, RNA, and proteins. Reserved for steroid failure or unacceptable adverse effects from prolonged steroid use; can be used for steroid-sparing effects to lower steroid dose.

Adult

2-3 mg/kg/d PO divided ac; adjust dose prn
Some experts advocate increasing dose until MCV >100 fL; do not increase dose if leukopenia develops

Pediatric

Not established

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

Pregnancy

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

Precautions

Increases risk of neoplasia; caution in liver disease and renal impairment; rarely causes hepatotoxicity (2-3 fold elevation of hepatic enzymes common); hematologic toxic effects include dose-related leukopenia, thrombocytopenia, macrocytic anemia

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
Multimedia: Temporal/Giant Cell Arteritis
References
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Further Reading

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Keywords

cranial arteritis, giant cell arteritis, GCA, granulomatous arteritis, Horton syndrome, polymyalgia arteritica, polymyalgia rheumatica, polymyalgia, temporal arteritis, anterior ischemic optic neuropathy, AION

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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  Honoraria Consulting; Teva Marion Honoraria Consulting; 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.

Medical Editor

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.

Pharmacy Editor

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

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

Chief Editor

Michael K Racke, MD, Professor of Neurology and Molecular Virology, Immunology, and Medical Genetics, Chairman of Neurology, Chief of Neurology Service, Ohio State University Medical Center
Michael K Racke, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association for the Advancement of Science, American Association of Immunologists, and American Neurological Association
Disclosure: Teva Neuroscience Consulting fee Consulting; Peptimmune Inc. Consulting fee Consulting; Bristol Myers Squibb Consulting fee Consulting; EMD Serono Honoraria Speaking and teaching

 
 
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