eMedicine Specialties > Rheumatology > Vasculitis

Giant Cell Arteritis: Follow-up

Author: Mythili Seetharaman, MD, Consulting Staff, Einstein Arthritis Center, Albert Einstein Medical Center, St Christopher's Hospital for Children
Coauthor(s): Stephen A Paget, MD, Chair, Division of Rheumatology, Chief, Department of Medicine, Hospital for Special Surgery; Professor, Department of Internal Medicine, Division of Rheumatology, Weill-Cornell University; Evan Leibowitz, MD, Fellow, Department of Internal Medicine, Division of Rheumatology, Valley Hospital
Contributor Information and Disclosures

Updated: Nov 14, 2008

Follow-up

Further Outpatient Care

  • Symptom control
    • Relapse of giant cell arteritis (GCA) is common (25-60%).
    • In almost all patients, the steroid dosage can be significantly decreased; however, the dose may need to be briefly increased then decreased again because of the ebb and flow of the inflammatory process.
    • The duration of treatment of polymyalgia rheumatica varies significantly among patients, with some needing corticosteroids for only 1 year and others for more than 5 years. In general, polymyalgia rheumatica is a self-limited disorder that lasts approximately 2 years, but some patients may also need low doses of prednisone for a frustratingly longer period. Constantly try to optimally balance the positive and negative effects of steroids in elderly patients with many comorbid disorders.
    • Following symptom control, reduce the dosage of corticosteroids to the lowest level required to suppress symptoms because the cumulative morbidity associated with long-term therapy often exceeds that of the underlying disease.
    • Increase the dosage of prednisone only if symptoms recur, not based solely on an elevation of the ESR. An elevated ESR without accompanying symptoms or signs of polymyalgia rheumatica or giant cell arteritis could be related to an infection.
    • If the patient improves (as expected) but the ESR does not improve or rises after a prior fall, look for another cause of an elevated ESR (eg, infection). Although uncommon, discontinuing steroid therapy after less than 2 years is possible.
    • Patients with polymyalgia rheumatica may require low-dose steroids (eg, <5 mg/d of prednisone) for 6 months to 5 years.
    • In patients with giant cell arteritis, maintain high-dose steroid therapy only long enough for symptoms to resolve and then taper to a maintenance dosage of prednisone over several months. Both clinical signs and the ESR (or CRP) assist in monitoring the patient's response.
    • Patients with visual involvement usually require slower tapering of corticosteroids. While the average patient requires continued maintenance therapy with prednisone for 2 years, some patients may need treatment for as long as 5 years. Because the incidence of new visual damage appears to decrease with disease duration, consider a repeat temporal artery biopsy before restarting corticosteroids in patients who relapse after 18-24 months.
  • Long-term therapy
    • Narvaez et al retrospectively studied the effect of long-term therapy on patients with polymyalgia rheumatica and patients with giant cell arteritis (ie, 10-y period).24 Among patients with pure polymyalgia rheumatica, 49% were off steroids in a mean duration of 23 months and remained symptom-free at 11 months of follow-up. These patients had a higher remission rate than those with polymyalgia rheumatica associated with giant cell arteritis.
    • In the patients with polymyalgia rheumatica associated with giant cell arteritis, 29% were off steroids after a mean duration of 31 months and remained symptom-free at 14 months of follow-up. The median duration of therapy was 56 months for this group; 50% of these patients required treatment for more than 4 years.
    • Factors that predict the need for prolonged therapy and increased relapse risk are older age at diagnosis, female sex, higher baseline ESR, and rapid taper of prednisone.
  • Prophylactic measures
    • Given the high risk of corticosteroid-induced osteoporosis, obtain a baseline bone densitometry value at the start of therapy for polymyalgia rheumatica or giant cell arteritis and administer an aggressive bone-preserving regimen accordingly.
    • Ensure that all patients are on 1500 mg of calcium and 800 IU of vitamin D-3 per day.
    • Consider adding osteoporosis preventive therapy with bisphosphonates, calcitonin, or hormonal therapy if the baseline bone densitometry findings show osteopenia or osteoporosis.
    • Administer age-appropriate vaccinations (eg, influenza vaccinations, pneumococcal vaccine polyvalent [Pneumovax]).
    • Low-dose aspirin (81 mg enteric coated) may be used for prevention of visual loss.

Complications

  • Loss of vision
  • Scalp necrosis (rare)
  • Cerebrovascular accidents (rare)
  • Aortic aneurysm, dissection, or stenosis
  • Aortic arch syndromes (rare)
  • Coronary involvement (rare)
  • Corticosteroid-related complications
  • Synovitis
    • The presence of synovitis may complicate differentiation of polymyalgia rheumatica from rheumatoid arthritis. In addition, these disorders may occur in the same patient, adding further confusion.
    • Findings from radionuclide scans, imaging studies, and synovial biopsies demonstrate the presence of synovitis in proximal joints.

Prognosis

  • Polymyalgia rheumatica: This is generally a self-limited 2-year disorder and rarely progresses to giant cell arteritis.
  • Giant cell arteritis: Visual damage is often irreversible. The average duration of treatment is 2 years; however, some patients require treatment for 5 years or more. Morbidity from steroid therapy is often worse than the underlying disease.

Patient Education

  • Education is the most important step in allowing the patient to appreciate the clinical facets of this illness, the potential adverse effects of the therapy, and the need for monitoring.

Miscellaneous

Medicolegal Pitfalls

  • Failure to consider the following:
    • Corticosteroid-related adverse effects
    • Long-term use of high-dose corticosteroid therapy due to the treatment of an elevated ESR in an improved patient
    • Avascular necrosis, cataracts, or osteoporosis with vertebral or peripheral fracture
  • Many physicians (eg, ophthalmologists; ear, nose, and throat specialists; neurologists; primary care physicians) may observe patients with giant cell arteritis, and their failure to diagnose giant cell arteritis because they either do not think about it until too late or feel that a normal or low ESR excludes the possibility may lead to blindness in the patient.
 


More on Giant Cell Arteritis

Overview: Giant Cell Arteritis
Differential Diagnoses & Workup: Giant Cell Arteritis
Treatment & Medication: Giant Cell Arteritis
Follow-up: Giant Cell Arteritis
References
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Further Reading

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Keywords

giant cell arteritis, temporal arteritis, GCA, cranial arteritis, polymyalgia rheumatica, PMR, occult giant cell arteritis, aching syndrome, Horton disease, Horton's disease, intimal hyperplasia, vasculopathy, ischemic optic retinopathy, stenotic lesions, ischemic optic retinopathy, vision loss, visual defects, fever of unknown origin, systemic vasculitis, granulomatous arteritis, limb claudication

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Contributor Information and Disclosures

Author

Mythili Seetharaman, MD, Consulting Staff, Einstein Arthritis Center, Albert Einstein Medical Center, St Christopher's Hospital for Children
Mythili Seetharaman, MD is a member of the following medical societies: American College of Rheumatology and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Stephen A Paget, MD, Chair, Division of Rheumatology, Chief, Department of Medicine, Hospital for Special Surgery; Professor, Department of Internal Medicine, Division of Rheumatology, Weill-Cornell University
Stephen A Paget, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Evan Leibowitz, MD, Fellow, Department of Internal Medicine, Division of Rheumatology, Valley Hospital
Evan Leibowitz, MD is a member of the following medical societies: Alpha Omega Alpha and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

John Varga, MD, Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University
John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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