title: Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature.
abstract: OBJECTIVE
  To determine the frequencies and types of ophthalmologic manifestations in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN) and ANCA-associated vasculitides (granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA); Churg-Strauss syndrome (CSS)) and review the literature on eye involvement in these diseases.
  
  
  METHODS
  This retrospective analysis was conducted on the ophthalmologic manifestations of SNV patients entered into the French Vasculitis Study Group database between July 1955 and August 2008.
  
  
  RESULTS
  Among the 1286 identified patients, 214 (16.6%) had ophthalmologic manifestations at diagnosis, significantly more often in GPA (117/343, 34.1%) than in EGPA (30/270, 11.1%; P = 0.0001), PAN (42/393, 10.7%; P = 0.0001) or MPA (25/280, 8.9%; P = 0.0001). The 3 most common recorded ophthalmologic manifestations were conjunctivitis (89, (7%)), episcleritis (56, (4%)), and/or blurred vision (44, (3%)), mainly caused by retinal vasculitis in 5, oculomotor nerve palsy in 4, uveitis in 4 and/or optic neuropathy in 3. Orbital inflammatory tumor, another common feature was rather specific to GPA (23/349, 6.6% (P = 0.0001)) compared to other SNV. The literature on ophthalmologic manifestations of SNV is limited to case reports except for GPA, in which the eye involvement frequency ranged from 29% to 57%.
  
  
  CONCLUSIONS
  Eye manifestations were more common in GPA than MPA, PAN and EGPA, but can be sight-threatening in any SNV. Given the heterogeneity of ophthalmologic involvement in SNV, close collaboration between the ophthalmologists and internists is critical.
authors.affiliation: Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Pierre-Raphaël
authors.initials: PR
authors.lastname: Rothschild
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Raphaele
authors.initials: R
authors.lastname: Seror
authors.forename: Antoine P
authors.initials: AP
authors.lastname: Brézin
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Delair
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000175:diagnosis*; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis; D004803:Eosinophilic Granuloma / Q000150:complications / Q000175:diagnosis*; D005123:Eye / Q000473:pathology; D005128:Eye Diseases / Q000175:diagnosis* / Q000209:etiology; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis; D006801:Humans; D055953:Microscopic Polyangiitis / Q000150:complications / Q000175:diagnosis; D009336:Necrosis / Q000150:complications / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies