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Classification of disease severity in retinitis pigmentosa
  1. Mustafa Iftikhar1,
  2. Marili Lemus1,
  3. Bushra Usmani2,
  4. Peter A Campochiaro1,
  5. José Alain Sahel2,
  6. Hendrik P N Scholl1,3,4,
  7. Syed Mahmood Ali Shah1,2
  1. 1 The Wilmer Eye Institute, School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA
  2. 2 Department of Ophthalmology, School of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  3. 3 Institute of Molecular and Clinical Ophthalmology Basel (IOB), Basel, Switzerland
  4. 4 Department of Ophthalmology, University of Basel, Basel, Switzerland
  1. Correspondence to Dr Syed Mahmood Ali Shah, Department of Ophthalmology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA; sms{at}


Aim To develop a simple and easily applicable classification of disease severity in retinitis pigmentosa (RP).

Methods This is a retrospective cross-sectional study. Visual acuity (VA), visual field width (VF) and ellipsoid zone width (EZ) were obtained from medical records of patients with RP. A scoring criterion was developed wherein each variable was assigned a score from 0 to 5 depending on its distribution. The cumulative score (from 0 to 15) was used to classify disease severity from grade 0 to 5. The scores were correlated with each other and the final grade. The grades were then correlated with age and disease duration.

Results The median age (range) of patients (n=93) was 55 (12–87) years, 51% were female, 70% had been diagnosed within 10 years, and 50% had autosomal recessive disease. Most eyes (n=181) at least had a VA of 20/40 (67%), a VF of 20° (75%) and an EZ of 5° (76%). All scores were correlated with each other (r=0.509–0.613; p<0.001 for all) and with the final grade (r=0.790–0.869; p<0.001 for all). Except for grade 0 (5%), all grades were evenly distributed: 21% for grade 1, 23% for grade 2, 22% for grade 3, 17% for grade 4 and 12% for grade 5. Grades were correlated with both age (r=0.252; p<0.001) and disease duration (r=0.383; p<0.001).

Conclusions We present a simple, objective and easy to use disease severity classification for RP which can be used to categorise and compare patients.

  • retinitis pigmentosa
  • classification
  • severity

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  • Contributors All authors fulfil the ICMJE guidelines for authorship including substantial contributions to the study design, interpretation of data and drafting the work for important intellectual content. All authors were involved in the final approval of the version to be published and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. SMAS accepts full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The study was approved by the Institutional Review Board of the Johns Hopkins Medical Institutions and was conducted in accordance with the tenets of the Declaration of Helsinki.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement All data relevant to the study are included in the article or uploaded as online supplementary information.

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