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Giant-Cell Arteritis and Polymyalgia Rheumatica

Cornelia M. Weyand, MD; and Jörg J. Goronzy, MD
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From the Mayo Clinic, Rochester, Minnesota.

Note: A discussion of the molecular and cellular biology of vasculitis can be found in Weyand CM, Goronzy JJ. Medium- and large-vessel vasculitis. N Engl J Med. 2003; 349:160-9.

Grant Support: By grants from the National Institutes of Health (R01 AI44142, R01 AR42527, R01 EY11916, R01 HL 63919, R01 AG15043, and R01 AR41974) and by the Mayo Foundation.

Potential Financial Conflicts of Interest: None disclosed.

Requests for Single Reprints: C.M. Weyand, MD, Mayo Clinic, Guggenheim 401, 200 First Street SW, Rochester, MN 55905; e-mail, weyand.cornelia@mayo.edu.

Current Author Addresses: Drs. Weyand and Goronzy: Mayo Clinic, Guggenheim 401, 200 First Street SW, Rochester, MN 55905.

Ann Intern Med. 2003;139(6):505-515. doi:10.7326/0003-4819-139-6-200309160-00015
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Giant-cell arteritis is an immune-mediated disease characterized by granulomatous infiltrates in the wall of medium-size and large arteries. The immunopathology consists of 2 components. Excessive cytokine production (for example, of interleukin-1 and interleukin-6) induces systemic inflammation with an exuberant acute-phase response. In parallel, interferon-, which is released by T cells captured in the arterial wall, activates tissue-injurious macrophages. In response to the immune injury, the artery generates hyperplasia of the intima that leads to luminal occlusion and subsequent tissue ischemia. Despite the systemic character of the disease, distinct vascular territories are preferentially affected. On the basis of the predominant involvement, clinical subtypes can be distinguished: cranial giant-cell arteritis with ischemic complications in the eye, the face, and the central nervous system; large-vessel giant-cell arteritis with occlusions in the subclavian or axillary vessels; aortic giant-cell arteritis; giant-cell arteritis presenting as an intense systemic inflammatory syndrome with nonstenosing vasculitis; and isolated polymyalgia rheumatica with myalgias, systemic inflammation, and subclinical vasculitis. Temporal artery biopsy remains the diagnostic procedure of choice to detect arteritis in cranial vessels. In other vascular territories, giant-cell arteritis is most commonly diagnosed by vascular imaging. Laboratory studies characteristically document the marked elevations of nonspecific acute-phase reactants, such as C-reactive protein and erythrocyte sedimentation rate. Cytokines, such as interleukin-6, that induce the acute-phase reaction are currently being explored as more sensitive biological markers of disease activity. Corticosteroids are highly effective in suppressing systemic inflammation, but they do not eliminate the immune responses in the vessel wall. In general, the clinical outcome of giant-cell arteritis is excellent, and efforts must now concentrate on tailoring therapies to the needs of the individual patient.


Grahic Jump Location
Figure 2.
The systemic inflammatory response in giant-cell arteritis and polymyalgia rheumatica.IL

Vessel wall inflammation is preceded and accompanied by an intense acute-phase response. Circulating macrophages are activated and release interleukin ( )-1 and interleukin-6, critical inducers of a multiorgan reaction involving the liver, the central nervous system, the vascular system, the bone marrow, and the immune system. Hepatic acute-phase reactants are useful in the laboratory diagnosis of giant-cell arteritis and polymyalgia rheumatica. The systemic inflammatory response can exist in the absence of fully developed vasculitis, as in the case of polymyalgia rheumatica. CRP = C-reactive protein.

Grahic Jump Location
Grahic Jump Location
Figure 1.
Pathogenic mechanisms in the vascular lesions of giant-cell arteritis.IFN-

Activated T cells and macrophages form granulomatous reactions in the arterial wall. T-cell triggering occurs in the adventitia, where the vasa vasorum provide a port of entrance for inflammatory cells. Upon stimulation, T cells secrete interferon-γ ( γ), a cytokine regulating effector functions of macrophages recruited to the lesions. Recruited macrophages differentiate into distinct subsets of tissue-injurious effector cells and produce matrix metalloproteinases and reactive oxygen intermediates. Macrophages and multinucleated giant cells also provide growth factors and angiogenic factors that support the response-to-injury program of the artery. The artery's reaction is maladaptive and leads to the formation of lumen-occlusive intimal hyperplasia. DC = dendritic cell; GC = multinucleated giant cell; IL = interleukin; M = macrophage; MMP = matrix metalloproteinases; PDGF = platelet-derived growth factor; ROI = reactive oxygen intermediates; T = T cell; VEGF = vascular endothelial growth factor.

Grahic Jump Location




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Summary for Patients

Giant-Cell Arteritis and Polymyalgia Rheumatica

The full report is entitled “Giant-Cell Arteritis and Polymyalgia Rheumatica.” It is in the 16 September 2003 issue of Annals of Internal Medicine (volume 139, pages 505-515). The authors are C.M. Weyand and J.J. Goronzy.


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