Giant Cell Arteritis Workup

  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: May 23, 2012
 

Laboratory Studies

Blood studies

The laboratory hallmark of polymyalgia rheumatica and giant cell arteritis (GCA) is an elevation in the acute-phase reactants, ie, ESR and C-reactive protein (CRP). The ESR usually exceeds 50 mm/h and may exceed 100 mm/h. However, an ESR in the low 30s or 20s does not exclude polymyalgia rheumatica or giant cell arteritis if other characteristic clinical or, in the case of giant cell arteritis, pathological, features are present.[23, 24]

Lopez-Diaz et al (2008) found that visual ischemic events were more common in patients with an ESR in the range of 70-100 mm/h at the time of diagnosis.[25]

Normocytic normochromic anemia and thrombocytosis occur in approximately 50% of patients with polymyalgia rheumatica and are good guides to the state of inflammation. These findings are also common in giant cell arteritis. Anemia has been shown to have a good negative predictive value for severe ischemic complications in giant cell arteritis.[26]

In both polymyalgia rheumatica and giant cell arteritis, the frequency of rheumatoid factor, antinuclear antibodies, and other autoreactive antibodies is not higher than that of age-matched controls. Complement levels are normal, and cryoglobulins and monoclonal immunoglobulins are absent.

Findings from liver function tests, especially the alkaline phosphatase level, may be elevated in approximately one third of patients, most commonly in giant cell arteritis rather than pure polymyalgia rheumatica.

Muscle enzyme levels (eg, creatine kinase, aldolase) are normal in both polymyalgia rheumatica and giant cell arteritis.

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Imaging Studies

Researchers have recently explored the role of color duplex ultrasonography in the diagnosis of giant cell arteritis. A hypoechoic halo around the temporal artery lumen on color duplex sonograms was reported in 22 of 30 patients (73%) with biopsy-proven giant cell arteritis.[27] The halo was observed bilaterally in 17 of these patients and disappeared at a mean of 16 days after the initiation of corticosteroid therapy. The presence of the halo had a sensitivity of 73% and was 100% specific for giant cell arteritis. The halo represents edema in the artery wall. Although some centers consider this finding a major decision-maker in their diagnostic equation, others do not believe that such a halo is definitive for giant cell arteritis. Superficial temporal artery biopsy remains the standard for diagnosis of giant cell arteritis (see Procedures).

In patients with giant cell arteritis, extracranial involvement can occur with or without symptoms. Further evaluation with MRI/MRA of the aortic branch could be considered to diagnose such involvement.[28]

Thoracic or abdominal ultrasonography may be helpful for diagnosing and monitoring patients with aortic aneurysms.

In giant cell arteritis, temporal artery arteriography has no diagnostic value and does not aid in predicting the proper biopsy site for the temporal artery.

Polymyalgia rheumatica has no characteristic imaging abnormalities. However, recent MRI findings of tendinitis and bursitis in the shoulders of patients with polymyalgia rheumatica support the soft tissues as significant sites of inflammation and sources of symptoms in polymyalgia rheumatica.

Positron emission tomography (PET) scan is showing promise in the diagnosis of giant cell arteritis in some case reports.[29]

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Other Tests

  • Electromyographic studies: Results are within normal limits. This study is rarely needed in patients with a clinical presentation of polymyalgia rheumatica.
  • Synovial fluid and tissue studies: Leukocyte counts in joint fluid, which are rarely available for study, range from 1000-8000/µL, with a preponderance of mononuclear cells. Synovial biopsy findings, when available, reveal mild synovial proliferation with slight lymphocytic infiltration.[30]
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Procedures

Muscle biopsy

Histology findings are not diagnostic in polymyalgia rheumatica; type II muscle fiber atrophy probably represents disuse.

This study is not indicated because patients with polymyalgia rheumatica do not present with weakness or muscle enzyme abnormalities.

Temporal artery biopsy

Consider this biopsy if a patient with polymyalgia rheumatica has symptoms or signs suggestive of giant cell arteritis or is unresponsive to 15 mg prednisone daily. It may also be indicated in the workup of an elderly patient with fever of unknown origin with a high ESR in whom infection and malignancy testing has been unrevealing.

Temporal artery biopsy can be performed on an ambulatory basis and, if findings are positive, can dramatically diminish the diagnostic studies otherwise needed in evaluating systemically ill patients with an elevated ESR.

Several clinical studies demonstrate that the likelihood of positive findings after temporal artery biopsy in patients with giant cell arteritis polymyalgia rheumatica is greatly enhanced if temporal artery pulses are absent or diminished, even in the absence of other localizing signs. The presence of a headache and jaw claudication may also increase the yield.[31]

No difference is evident in the degree of ESR elevation, the presence of minor visual symptoms, sex, age, or the duration of symptoms among patients with polymyalgia rheumatica who do not have giant cell arteritis; furthermore, approximately 10% of patients with polymyalgia rheumatica and localized temporal artery signs have negative biopsy findings.

Strategies for planning temporal artery biopsies in patients suggested to have giant cell arteritis include the following:

  • Always perform the biopsy on the temporal artery on the symptomatic side of the head.
  • If a specific part of the artery is tender, beaded, or inflamed, include that area in the biopsy. No information exists on whether the artery trunk or a distal branch specimen is best. Take at least 2-3 cm of the artery.[32] Make multiple sections because the process may be segmental.
  • Studies performed during the 1970s and 1980s show that bilateral temporal artery biopsies can increase the sensitivity of diagnosing giant cell arteritis by 11-60%. However, recent studies have found that bilateral biopsies do not increase the diagnostic yield in the vast majority of patients (99%).[33, 34] However, questions have been raised about the use of steroid treatment for giant cell arteritis in the absence of positive biopsy results.[35]
  • Results of temporal artery biopsies remain positive for characteristic giant cell arteritis pathology after as long as 4 weeks while high doses of corticosteroids are administered. Thus, the physician should never delay the institution of steroids because of fear of ruining the chances of finding an inflamed artery on a biopsy sample.
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Histologic Findings

An inflammatory infiltrate, predominantly of mononuclear cells, usually involves the entire vessel wall (ie, panarteritis). Fragmentation of the internal elastic lamina is characteristic. Fibrinoid necrosis is not a feature of the lesion. Giant cells are commonly present, and they often seem to engulf parts of the internal elastic lamina. The giant cells are difficult to find in some cases, and their absence does not exclude the diagnosis. Intimal proliferation is often marked, is a nonspecific feature in this age group, and does not suggest past or present arteritis if found alone. When giant cell arteritis involves larger vessels, the lesions are indistinguishable from those observed in Takayasu arteritis.

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Contributor Information and Disclosures
Author

Mythili Seetharaman, MD  Consultant Rheumatologist, OAA, Allentown, PA; Clinical Assistant Professor, Thomas Jefferson University Hospital; 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: Abbott pharmaceuticals Honoraria Speaking and teaching

Coauthor(s)

Stephen A Paget  MD, Physician in Chief Emeritus, Joseph P Routh Professor of Medicine, New York Hospital Weill Cornell Medical College; Program Director, Cornell Arthritis and Multipurpose Arthritis and Musculoskeletal Diseases Center (MAMDC), Hospital for Special Surgery

Stephen A Paget 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: Crescendo bioscience Consulting fee Consulting

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.

Specialty Editor Board

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.

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

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of 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 for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Alex J Mechaber, MD, FACP  Senior 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  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh 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: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

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