title: Multimodal imaging of the disease progression of birdshot chorioretinopathy.
abstract: OBJECTIVE
  To study outer retinal deterioration in relation to clinical disease activity in patients with birdshot chorioretinopathy using fundus autofluorescence and spectral-domain optical coherence tomography (OCT).
  
  
  METHODS
  A single-centre retrospective cohort study was carried out on 42 eyes of 21 patients with birdshot disease, using a multimodal imaging approach including fundus autofluorescence, OCT, fluorescein angiography and indocyanine green angiography in combination with a patient chart review. The patients' overall clinical activity of retinal vasculitis during the follow-up period was determined by periods of clinical activity as indicated by fluorescein angiography and associated treatment decisions. Image analysis was performed to examine the spatial correspondence between autofluorescence changes and disruption of the photoreceptor inner segment ellipsoid zone on OCT.
  
  
  RESULTS
  Three common types of outer retinal lesions were observed in fovea-centred images of 43% of patients: circular patches of chorioretinal atrophy, ellipsoid zone disruption on OCT, and outer retinal atrophy on autofluorescence and OCT. There was good spatial correspondence between ellipsoid zone disruption and areas of diffuse hyper-autofluorescence outside the fovea. Interestingly, the ellipsoid zone disruption recovered in four out of seven patients upon intensified therapeutic immunosuppression.
  
  
  CONCLUSIONS
  Most patients only developed peripapillary atrophy and occasional perivascular hypo-autofluorescence. A multimodal imaging approach with autofluorescence imaging and OCT may help to detect ellipsoid zone disruption in the central retina of patients with birdshot disease. Our results suggest that ellipsoid zone disruption may be related to both the activity and duration of retinal vasculitis, and could help to determine therapeutic success in birdshot disease.
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Michel M
authors.initials: MM
authors.lastname: Teussink
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Paulien I
authors.initials: PI
authors.lastname: Huis In Het Veld
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Lieuwe A M
authors.initials: LA
authors.lastname: de Vries
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Carel B
authors.initials: CB
authors.lastname: Hoyng
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: B Jeroen
authors.initials: BJ
authors.lastname: Klevering
authors.affiliation: Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Thomas
authors.initials: T
authors.lastname: Theelen
mesh_terms: D000328:Adult; D000368:Aged; D000080365:Birdshot Chorioretinopathy; D002825:Chorioretinitis / Q000145:classification / Q000000981:diagnostic imaging* / Q000503:physiopathology; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D061848:Optical Imaging; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology
keywords: birdshot chorioretinopathy; fundus autofluorescence; longitudinal study; optical coherence tomography; photoreceptor; posterior uveitis