PMID- 28871101 OWN - NLM STAT- MEDLINE DCOM- 20190503 LR - 20220409 IS - 2045-2322 (Electronic) IS - 2045-2322 (Linking) VI - 7 IP - 1 DP - 2017 Sep 4 TI - Retrospective Analysis of Structural Disease Progression in Retinitis Pigmentosa Utilizing Multimodal Imaging. PG - 10347 LID - 10.1038/s41598-017-10473-0 [doi] LID - 10347 AB - In this report, we assess the natural progression rate of retinitis pigmentosa (RP) over an average of three years using spectral-domain optical coherence tomography (SD-OCT) and short wavelength fundus autofluorescence (SW-AF). Measurement of the ellipsoid zone (EZ) line width and hyperautofluorescent ring diameters was performed in 81 patients with RP in a retrospective, longitudinal fashion. Rate of structural disease progression, symmetry between eyes, and test-retest variability were quantified. We observed on average, EZ-line widths decreased by 140 microm (5.2%, p < 0.001) per year, and average horizontal and vertical hyperautofluorescent ring diameters decreased by 149 microm (3.6%, p < 0.001) and 120 microm (3.9%, p < 0.001) per year, respectively. The 95th percentile of this cohort had differences in progression slopes between eyes that were less than 154 microm, 118 microm, and 132 microm for EZ-line width and horizontal and vertical ring diameters, respectively. For all measures except horizontal ring diameter, progression rates were significantly slower at end-stage disease. From our data, we observed a statistically significant progression rate in EZ line width and SW-AF ring diameters over time, verifying the utility of these measurements for disease monitoring purposes. Additionally, calculated differences in progression slopes between eyes may prove useful for investigators evaluating the efficacy of unilateral treatments for RP in clinical trials. FAU - Cabral, Thiago AU - Cabral T AD - Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia University, New York, NY, USA. AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. AD - Department of Ophthalmology, Federal University of Espirito Santo, Vitoria, Brazil. AD - Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil. FAU - Sengillo, Jesse D AU - Sengillo JD AD - Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia University, New York, NY, USA. AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. AD - State University of New York Downstate Medical Center, Brooklyn, NY, USA. FAU - Duong, Jimmy K AU - Duong JK AD - Department of Biostatistics, Columbia University, New York, NY, USA. FAU - Justus, Sally AU - Justus S AD - Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia University, New York, NY, USA. AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. FAU - Boudreault, Katherine AU - Boudreault K AD - Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia University, New York, NY, USA. AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. AD - Department of Ophthalmology, University of Montreal, Montreal, QC, Canada. FAU - Schuerch, Kaspar AU - Schuerch K AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. FAU - Belfort, Rubens Jr AU - Belfort R Jr AD - Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil. FAU - Mahajan, Vinit B AU - Mahajan VB AD - Omics Laboratory, Byers Eye Institute, Department of Ophthalmology, Stanford University, Palo Alto, CA, USA. FAU - Sparrow, Janet R AU - Sparrow JR AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. AD - Department of Pathology & Cell Biology, Institute of Human Nutrition, College of Physicians and Surgeons, Columbia University, New York, NY, USA. FAU - Tsang, Stephen H AU - Tsang SH AD - Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Department of Ophthalmology, Columbia University, New York, NY, USA. Sht2@columbia.edu. AD - Edward S Harkness Eye Institute, New York-Presbyterian Hospital, New York, NY, USA. Sht2@columbia.edu. AD - Department of Pathology & Cell Biology, Institute of Human Nutrition, College of Physicians and Surgeons, Columbia University, New York, NY, USA. Sht2@columbia.edu. LA - eng GR - R21 AG050437/AG/NIA NIH HHS/United States GR - R01 EY018213/EY/NEI NIH HHS/United States GR - R01 EY024665/EY/NEI NIH HHS/United States GR - K08 EY020530/EY/NEI NIH HHS/United States GR - R01 EY026682/EY/NEI NIH HHS/United States GR - R01 EY024698/EY/NEI NIH HHS/United States GR - P30 EY019007/EY/NEI NIH HHS/United States GR - R01 EY025225/EY/NEI NIH HHS/United States GR - P30 CA013696/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20170904 PL - England TA - Sci Rep JT - Scientific reports JID - 101563288 RN - 0 (Biomarkers) SB - IM MH - Biomarkers MH - Disease Progression MH - Fluorescein Angiography MH - Humans MH - *Multimodal Imaging/methods MH - Reproducibility of Results MH - Retina/diagnostic imaging/metabolism/pathology MH - Retinitis Pigmentosa/*diagnostic imaging/metabolism/*pathology MH - Retrospective Studies MH - Severity of Illness Index MH - Tomography, Optical Coherence PMC - PMC5583352 COIS- The authors declare that they have no competing interests. EDAT- 2017/09/06 06:00 MHDA- 2019/05/06 06:00 PMCR- 2017/09/04 CRDT- 2017/09/06 06:00 PHST- 2017/02/03 00:00 [received] PHST- 2017/08/10 00:00 [accepted] PHST- 2017/09/06 06:00 [entrez] PHST- 2017/09/06 06:00 [pubmed] PHST- 2019/05/06 06:00 [medline] PHST- 2017/09/04 00:00 [pmc-release] AID - 10.1038/s41598-017-10473-0 [pii] AID - 10473 [pii] AID - 10.1038/s41598-017-10473-0 [doi] PST - epublish SO - Sci Rep. 2017 Sep 4;7(1):10347. doi: 10.1038/s41598-017-10473-0.