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The Role of Varicella Zoster Virus in Giant Cell Arteritis

  • Maria A. Nagel
  • Don Gilden
Chapter

Abstract

Giant cell arteritis (GCA), the most common systemic vasculitis in the elderly, is characterized by excruciating unilateral headache/head pain. The temporal artery (TA) is often nodular and tender. Many patients with GCA have jaw claudication and a history of polymyalgia rheumatica. Erythrocyte sedimentation rates and C-reactive protein are elevated. Diagnosis is confirmed by TA biopsy which reveals vessel wall damage and inflammation, with multinucleated giant cells and/or epithelioid macrophages in skip areas. The pathologies of GCA and that of intracerebral varicella zoster virus (VZV) vasculopathy are identical and characterized by granulomatous arteritis, suggesting that GCA represents an extracranial variant of VZV vasculopathy predominantly affecting the TA. Virological analysis of TAs from patients with GCA or from patients with clinically suspected GCA whose TA biopsies were pathologically negative revealed the presence of VZV in most TA biopsies, particularly in skip areas that correlate with adjacent GCA pathology or adventitial inflammation. The presence of VZV in GCA-positive and GCA-negative TAs implicates the virus in triggering the immunopathology of GCA and indicates the need for treatment with antiviral drugs in addition to corticosteroids in both groups of patients. Whether oral antiviral agents and steroids are as effective as intravenous acyclovir and steroids remains to be determined, as does the dosage and duration of treatment.

Abbreviations

APD

Afferent pupillary defect

CSF

Cerebrospinal fluid

GCA

Giant cell arteritis

HSV-1

Herpes simplex virus type 1

ION

Ischemic optic neuropathy

OD

Right eye

OS

Left eye

TA

Temporal artery

VZV

Varicella zoster virus

Notes

Acknowledgments

Supported in part by National Institutes of Health grants NS093716 (Gilden), AG032958 (Gilden and Nagel), and NS094758 (Nagel). The authors thank Marina Hoffman for editorial review and Cathy Allen for manuscript preparation.

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Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  1. 1.Department of NeurologyUniversity of Colorado School of MedicineAuroraUSA
  2. 2.Departments of Neurology and Immunology and MicrobiologyUniversity of Colorado School of MedicineAuroraUSA

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