title: Multimodal Imaging of Central Retinal Disease Progression in a 2-Year Mean Follow-up of Retinitis Pigmentosa.
abstract: OBJECTIVE
  To determine the rate of progression and optimal follow-up time in patients with advanced-stage retinitis pigmentosa (RP) comparing the use of fundus autofluorescence imaging and spectral-domain optical coherence tomography.
  
  
  METHODS
  Retrospective analysis of progression rate.
  
  
  METHODS
  Longitudinal imaging follow-up in 71 patients with retinitis pigmentosa was studied using the main outcome measurements of hyperautofluoresent ring horizontal diameter and vertical diameter along with ellipsoid zone line width from spectral-domain optical coherence tomography. Test-retest reliability and the rate of progression were calculated. The interaction between the progression rates was tested for sex, age, mode of inheritance, and baseline measurement size. Symmetry of left and right eye progression rate was also tested.
  
  
  RESULTS
  Significant progression was observed in >75% of patients during the 2-year mean follow-up. The mean annual progression rates of ellipsoid zone line and hyperautofluorescent ring horizontal diameter and vertical diameter were 0.45 degree (4.9%), 0.51 degree (4.1%), and 0.42 degree (4.0%), respectively. The ellipsoid zone line width and hyperautofluorescent ring horizontal diameter and vertical diameter had low test-retest variabilities of 8.9%, 9.5%, and 9.6%, respectively. This study is the first to demonstrate asymmetrical structural progression rate between right and left eye, which was found in 19% of patients. The rate of progression was significantly slower as the disease approached the fovea, supporting the theory that RP progresses in an exponential fashion. No significant interaction between progression rate and patient age, sex, or mode of inheritance was observed.
  
  
  CONCLUSIONS
  Fundus autofluorescence and optical coherence tomography detect progression in patients with RP reliably and with strong correlation. These parameters may be useful alongside functional assessments as the outcome measurements for future therapeutic trials. Follow-up at 1-year intervals should be adequate to efficiently detect progression.
authors.affiliation: Bernard & Shirlee Brown Glaucoma Laboratory and Barbara & Donald Jonas Stem Cell Laboratory, Department of Ophthalmology, Columbia University, New York, New York; Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Tharikarn
authors.initials: T
authors.lastname: Sujirakul
authors.affiliation: College of Physicians & Surgeons, Columbia University, New York, New York.
authors.forename: Michael K
authors.initials: MK
authors.lastname: Lin
authors.affiliation: Department of Biostatistics, Columbia University, New York, New York.
authors.forename: Jimmy
authors.initials: J
authors.lastname: Duong
authors.affiliation: Department of Biostatistics, Columbia University, New York, New York.
authors.forename: Ying
authors.initials: Y
authors.lastname: Wei
authors.affiliation: Department of Biostatistics, Columbia University, New York, New York.
authors.forename: Sara
authors.initials: S
authors.lastname: Lopez-Pintado
authors.affiliation: Bernard & Shirlee Brown Glaucoma Laboratory and Barbara & Donald Jonas Stem Cell Laboratory, Department of Ophthalmology, Columbia University, New York, New York; Department of Pathology and Cell Biology and Institute of Human Nutrition, Columbia University, New York, New York. Electronic address: sht2@columbia.edu.
authors.forename: Stephen H
authors.initials: SH
authors.lastname: Tsang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D018450:Disease Progression; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D064847:Multimodal Imaging; D012160:Retina / Q000473:pathology*; D055214:Retinal Photoreceptor Cell Outer Segment; D012174:Retinitis Pigmentosa / Q000175:diagnosis* / Q000235:genetics; D041623:Tomography, Optical Coherence; D014792:Visual Acuity; D058609:Visual Field Tests; D014794:Visual Fields; D055815:Young Adult