Clinical Rheumatology

, Volume 27, Issue 10, pp 1333–1336 | Cite as

Orbital Wegener’s granulomatosis: a case report and review of the literature

Case Report

Abstract

Wegener’s granulomatosis (WG) is a multisystem granulomatous, necrotizing vasculitis of presumed autoimmune origin that affects small- to medium-sized blood vessels. The respiratory tract and kidneys are typically involved (Gross and Reinhold-Keller, “Clinical features of primary ANCA-associated vasculitis” in Oxford textbook of rheumatology, third edition, 2004). The limited form usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease (Cassan et al., Am. J. Med. 49:366–379, 1970). Ocular involvement, which may be the initial manifestation, is often encountered and can result in significant morbidity and possibly blindness (Pakrou et al., Semin. Arthritis Rheum. 35:284–292, 2006). We report an unusual case of WG presenting as an orbital mass. The diagnostic triad of granulomatous inflammation with multinucleated giant cells, vasculitis, and necrosis was discovered on histopathology (McDonald and Edwards, JAMA 173:1205–1209, 1960).

Keywords

Eye Ocular Ophthalmic Orbital Vasculitis Wegener’s granulomatosis 

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Copyright information

© Clinical Rheumatology 2008

Authors and Affiliations

  1. 1.University of Connecticut Health CenterFarmingtonUSA
  2. 2.Hartford Hospital/Connecticut Multispecialty GroupHartfordUSA

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