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Original Investigation
September 2018

Development and Evaluation of Diagnostic Criteria for Vogt-Koyanagi-Harada Disease

Author Affiliations
  • 1The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Ophthalmology and Chongqing Eye Institute, Chongqing, People’s Republic of China
  • 2State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, People’s Republic of China
  • 3The Eye and ENT Hospital of Fudan University, Shanghai, People’s Republic of China
  • 4Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China
  • 5Beijing Tongren Eye Center, Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing Tongren Hospital, Capital Medical University, Beijing, People’s Republic of China
  • 6Department of Ophthalmology, Beijing Chao-Yang Hospital of Capital Medical University, Beijing, People’s Republic of China
  • 7Department of Ophthalmology, Peking University First Hospital, Beijing, People’s Republic of China
  • 8Department of Ophthalmology, Ningxia People’s Hospital, Yinchuan, People’s Republic of China
  • 9Department of Ophthalmology, the Second Xiangya Hospital of Central South University, Changsha, People’s Republic of China
  • 10Department of Ophthalmology, the First Affiliated Hospital of Harbin Medical University, Harbin, People’s Republic of China
  • 11Department of Ophthalmology, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, People’s Republic of China
  • 12Department of Ophthalmology, the Second Hospital of Anhui Medical University, Hefei, People’s Republic of China
  • 13Tianjin Medical University Eye Hospital, Tianjin, People’s Republic of China
  • 14The Eye Hospital of Wenzhou Medical University, Wenzhou, People’s Republic of China
  • 15Department of Ophthalmology, Shenzhen Children’s Hospital, Shenzhen, People’s Republic of China
  • 16Shenzhen Eye Hospital, Shenzhen Key Ophthalmic Laboratory, the Second Affiliated Hospital of Jinan University, Shenzhen, People’s Republic of China
  • 17Department of Ophthalmology, Jinling Hospital, Medical School of Nanjing University, Nanjing, People’s Republic of China
  • 18Department of Ophthalmology, the First Affiliated Hospital of Zhengzhou University, Henan Province Eye Hospital, Zhengzhou, People’s Republic of China
  • 19Department of Ophthalmology, Xi’an No. 4 Hospital, Xi’an, People’s Republic of China
  • 20Ophthalmic Center of the Second Hospital, Jilin University, Changchun, People’s Republic of China
  • 21Department of Ophthalmology, the Second People’s Hospital of Jinan City, Jinan, People’s Republic of China
  • 22Shanxi Eye Hospital, Taiyuan, People’s Republic of China
  • 23College of Information Engineering, Xiangtan University, Xiangtan, People’s Republic of China
  • 24University Eye Clinic Maastricht, Maastricht, Limburg, the Netherlands
JAMA Ophthalmol. 2018;136(9):1025-1031. doi:10.1001/jamaophthalmol.2018.2664
Key Points

Question  Can improvements be made in a currently used set of criteria to diagnose Vogt-Koyanagi-Harada (VKH) disease?

Findings  In a case-control study including 1257 patients, the Diagnostic Criteria for VKH Disease (DCV) were developed using latent class analysis on 634 patients with VKH disease and 623 patients with non-VKH uveitis. The DCV were compared with another set of samples containing 537 patients with definite VKH disease and 525 patients with non-VKH uveitis and showed a higher sensitivity and negative predictive value compared with the Revised Diagnostic Criteria for VKH Disease.

Meaning  These results suggest that use of the DCV could result in an improvement in the sensitivity and negative predictive value to diagnose VKH disease.

Abstract

Importance  To our knowledge, a set of well-defined diagnostic criteria is not yet developed for the diagnosis of Vogt-Koyanagi-Harada (VKH) disease.

Objective  To develop and evaluate a set of diagnostic criteria for VKH disease using data from Chinese patients.

Design, Setting, and Participants  This case-control study reviewed medical records of patients from a tertiary referral center between October 2011 and October 2016. Data from 634 patients with VKH disease and 623 patients with non-VKH uveitis from southern China were used to develop the Diagnostic Criteria for VKH Disease (DCV). Data from an additional group of 537 patients with a definite VKH disease diagnosis and 525 patients with non-VKH uveitis from northern China were used to evaluate the diagnostic criteria.

Main Outcomes and Measures  Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and receiver operating characteristic.

Results  Of the 1257 patients used to construct the DCV, 665 (52.9%) were male, and the mean (SD) age at disease onset was 38.6 (13.6) years. The 3-class model and 21 clinical findings were selected by latent class analysis. Variables with a high positive rate in the early-phase or late-phase VKH group or high specificity constituted essential parameters. Constellations of these essential parameters constructed the DCV. The sensitivity and NPV of the DCV were higher than those of the Revised Diagnostic Criteria for VKH Disease (RDC) (sensitivity: 94.6% vs 71.9%; difference, 22.7%; 95% CI, 18.5-27.0; NPV: 94.3% vs 76.6%; difference, 17.7%; 95% CI, 13.9-21.5). The specificity and PPV of the DCV were not different from that of the RDC (specificity: 92.2% vs 93.9%; difference, 1.7%; 95% CI, −1.4 to 4.8; PPV: 89.3% vs 92.3%; difference, 3.0%; 95% CI, −1.4 to 4.8). The area under the receiver operating characteristic curve of the DCV and the RDC were 0.934 (95% CI, 0.917-0.951) and 0.829 (95% CI, 0.803-0.855), respectively.

Conclusions and Relevance  The DCV were developed and evaluated using data from Chinese patients with VKH disease and showed a high sensitivity, NPV, and area under the receiver operating characteristic curve in comparison with the RDC. However, they were developed using a retrospective analysis and should be evaluated in prospective studies in other racial/ethnic populations.

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