title: Birdshot Retinochoroidopathy: Prognostic Factors of Long-term Visual Outcome.
abstract: OBJECTIVE
  To determine the prognostic factors of long-term visual outcome in birdshot retinochoroidopathy (BRC).
  
  
  METHODS
  Design: Retrospective case series. Study Population: Successive HLA-A29+ BRC patients whose latest visit was between May and August 2013 at a single tertiary center (Pitié-Salpétrière Hospital, Paris).
  
  
  METHODS
  Endpoint visual status (remission or deterioration) was determined for each patient based on clinical and ancillary data from the latest visit including optical coherence tomography (OCT), automated visual field (AVF), and angiograms. Main Outcome Measure: Epidemiologic, clinical, OCT, AVF, angiographic, and electrophysiological data at baseline were correlated to final visual status.
  
  
  RESULTS
  Fifty-five patients were included. Mean observation period was 8 years (range: 0.6-23 years). Mean disease duration was 9.8 years (range: 1.2-32.7 years). Female-to-male sex ratio was 1.6:1. Factors of good visual prognosis (remission vs deterioration) included at baseline: late age of disease onset (49.5 vs 45 years, P = .05), presence of vitreous inflammatory reactions >2+ (35.9% vs 6.2%, P = .04), vascular leakage on fluorescein angiograms (FA) (44.4% vs 12.5%, P = .03), absence of macular pigment epithelium atrophy on FA (88.9% vs 62.5%, P = .05), and presence of macular edema on OCT (33.3% vs 6.2%, P = .04). Preserved electrooculography light peak and Arden ratio (P = .06) and presence of choroidal spots on infracyanine green angiograms (80.0% vs 53.3%, P = .08) seemed associated with the best prognoses.
  
  
  CONCLUSIONS
  This study suggests a series of prognostic factors of long-term visual outcome in BRC. Keeping in mind the insidious evolution of the disease, knowledge of such prognostic factors should help tailor the treatment and monitoring of birdshot patients.
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France. Electronic address: saratouhami@gmail.com.
authors: Sara
authors: S
authors: Touhami
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France.
authors: Christine
authors: C
authors: Fardeau
authors: UPMC Univ. Paris 6, Department of Public Health; AP-HP, University Hospital Pitié-Salpêtrière Charles-Foix, Department of Biostatistics Public Health and Medical Informatics, Paris, France.
authors: Antoine
authors: A
authors: Vanier
authors: Ophthalmology Department, Centre Hospitalier Intercommunal de Créteil, Créteil, France.
authors: Olivia
authors: O
authors: Zambrowski
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France.
authors: Rafaela
authors: R
authors: Steinborn
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France.
authors: Claude
authors: C
authors: Simon
authors: UPMC Univ. Paris 6, Department of Public Health; AP-HP, University Hospital Pitié-Salpêtrière Charles-Foix, Department of Biostatistics Public Health and Medical Informatics, Paris, France; Sorbonne Universités, UPMC University Paris 06 UMR_S1136, and INSERM UMR_S 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, Paris, France.
authors: Sophie
authors: S
authors: Tezenas du Montcel
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France.
authors: Bahram
authors: B
authors: Bodaghi
authors: Ophthalmology Department, Reference Center in Rare Diseases, DHU Sight Restore, Hôpital Pitié Salpêtrière, University Paris VI, Paris, France.
authors: Phuc
authors: P
authors: Lehoang
mesh_terms: D000328:Adult; D000368:Aged; D000080365:Birdshot Chorioretinopathy; D002199:Capillary Permeability; D002825:Chorioretinitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D004585:Electrooculography; D005128:Eye Diseases / Q000175:diagnosis; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D015234:HLA-A Antigens / Q000276:immunology; D006801:Humans; D008269:Macular Edema / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis* / Q000503:physiopathology; D014792:Visual Acuity / Q000502:physiology*; D014794:Visual Fields / Q000502:physiology*; D014822:Vitreous Body / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D015234:HLA-A Antigens; C035524:HLA-A29 antigen