Clinical and imaging features predictive of orbital granulomatosis with polyangiitis and the risk of systemic involvement

Tan LT 1 , Davagnanam I 2 , Isa H 3 , Taylor SR 4 , Rose GE 5 , Verity DH 6 Show all authors , Pusey CD 7 , Lightman S 8

Affiliations 

  • 1 UCL Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom
  • 2 Moorfields Eye Hospital, London, United Kingdom; Brain Repair & Rehabilitation Unit, Institute of Neurology, London, United Kingdom
  • 3 UCL Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom; Department of Ophthalmology, Universiti Kebangsaan Malaysia, Selangor, Malaysia
  • 4 UCL Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom; Department of Ophthalmology, Imperial College London, London, United Kingdom
  • 5 UCL Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom
  • 6 Moorfields Eye Hospital, London, United Kingdom
  • 7 Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom; Department of Ophthalmology, Imperial College London, London, United Kingdom
  • 8 UCL Institute of Ophthalmology, London, United Kingdom; Moorfields Eye Hospital, London, United Kingdom; Multidisciplinary Vasculitis Clinic, Hammersmith Hospital, London, United Kingdom. Electronic address: [email protected]
Ophthalmology, 2014 Jun;121(6):1304-9.
PMID: 24560566 DOI: 10.1016/j.ophtha.2013.12.003

Abstract

Granulomatosis with polyangiitis (GPA), previously Wegener's granulomatosis, requires prompt diagnosis and systemic review to exclude life-threatening disease. However, early diagnosis of orbital GPA may be difficult because anti-neutrophil cytoplasmic antibody (ANCA) and anti-PR3 antibody screening can be negative at presentation and orbital biopsies taken for diagnosis may not show the classic features of GPA. This study was designed to compare GPA with other causes of orbital inflammation and to identify the presenting clinical and imaging features most likely to predict GPA and its systemic spread.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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