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Journal Article Ahead of print 1 citation

PubMed: 42560698 DOI: 10.1001/jamaophthalmol.2026.2957

Standardization of Retinal Vasculitis Terminology With Consensus Definitions From a Delphi Panel.

JAMA Ophthalmol

Authors: James T Rosenbaum, Sapna Gangaputra, Marc D de Smet, Douglas A Jabs, James R Stone, Ilknur Tugal-Tutkun, Liang Yao, Vishali Gupta, Rupesh Agrawal

Affiliations

  • Legacy Devers Eye Institute, Portland, Oregon.
  • Vanderbilt Eye Institute, Nashville, Tennessee.
  • Micro Invasive Ocular Surgery Clinic, Lausanne, Switzerland.
  • New York Eye and Ear Infirmary of Mount Sinai, Icahn School of Medicine, New York.
  • Center for Clinical Trials and Evidence Synthesis, Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.
  • Department of Ophthalmology, The Johns Hopkins Wilmer Eye Institute, Baltimore, Maryland.
  • Department of Pathology, Massachusetts General Hospital, Boston.
  • Department of Pathology, Harvard Medical School, Boston, Massachusetts.
  • Uveitis Service, Eye Protection Foundation, Bayrampasa Eye Hospital, Istanbul, Türkiye.
  • Department of Ophthalmology, Istanbul University, Istanbul, Türkiye.
  • Lee Kong Chian School of Medicine, Nanyang Technical University, Singapore.
  • Advanced Eye Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
  • Lee Kong Chian School of Medicine, Nanyang Technical University, Singapore. National Healthcare Group Eye Institute, Tan Tock Seng Hospital, Singapore. Singapore Eye Research Institute, Singapore. Eye ACP Program, Duke NUS Medical School, Singapore. Department of Ophthalmology, National University of Singapore, Singapore.

Abstract

IMPORTANCE

Retinal vasculitis is a sight-threatening condition associated with diverse ocular and systemic diseases. Variability in terminology and definitions across clinical practice and research has limited diagnostic consistency, communication among specialists, and comparability of studies.

OBJECTIVE

To develop standardized, consensus-based definitions for retinal vasculitis and related terms to improve diagnostic clarity and harmonize communication among ophthalmologists and other medical specialties.

DESIGN, SETTING, AND PARTICIPANTS

This was an international modified Delphi consensus study conducted using a structured consensus process guided by a comprehensive literature review, including systematic reviews and meta-analyses. Two rounds of Delphi surveys were performed, and consensus was predefined as at least 75% agreement. Included was an international expert panel of 27 specialists in ophthalmology, rheumatology, pathology, imaging, and research methodology. The specialists had recognized expertise in retinal and systemic vasculitis, retinal imaging, or consensus methodology. Study data were analyzed from March to September 2025.

EXPOSURE

Participation in a structured Delphi process evaluating proposed definitions and terminology related to retinal vasculitis and associated vascular inflammatory entities.

MAIN OUTCOMES AND MEASURES

Consensus definitions for retinal vasculitis and related entities, with agreement using predefined consensus thresholds.

RESULTS

A total of 27 international experts participated in the Delphi process. After 5 executive committee members involved in developing preliminary definitions were excluded from voting to minimize bias, a total of 22 independent experts completed both Delphi rounds. All candidate statements exceeded the predefined consensus threshold in the first round (agreement range, 83.3%-95.5%), although some demonstrated variability in the strength of agreement. After refinement of definitions, consensus strengthened in the second round, with agreement levels ranging from 95.2% to 100%. The panel established consensus definitions for 8 terms: retinal vasculitis, vascular leakage, infectious retinal vasculitis, noninfectious retinal vasculitis, retinal perivasculitis, retinal vasculopathy, primary retinal vasculitis, and far-peripheral vascular leakage. These definitions were developed to distinguish vascular leakage from true vasculitis, clarify inflammatory and noninflammatory vascular disorders, and promote consistent terminology across clinical practice and research.

CONCLUSIONS AND RELEVANCE

This international Delphi consensus established standardized nomenclature for retinal vasculitis and related vascular inflammatory entities. Adoption of these definitions may improve diagnostic accuracy, facilitate interpretation of imaging findings, enhance consistency across clinical studies, and support future research efforts, including the development of artificial intelligence-based classification systems for retinal vascular disease.

MeSH

Vasculitis, Retinal Vasculitis, Communication, Research, Eye Diseases, Retinaldehyde