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. 2021 Aug:228:205-211.
doi: 10.1016/j.ajo.2021.03.036. Epub 2021 Apr 9.

Classification Criteria for Vogt-Koyanagi-Harada Disease

Collaborators

Classification Criteria for Vogt-Koyanagi-Harada Disease

Standardization of Uveitis Nomenclature (SUN) Working Group. Am J Ophthalmol. 2021 Aug.

Abstract

Purpose: To determine classification criteria for Vogt-Koyanagi-Harada (VKH) disease.

Design: Machine learning of cases with VKH disease and 5 other panuveitides.

Methods: Cases of panuveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the panuveitides. The resulting criteria were evaluated on the validation set.

Results: One thousand twelve cases of panuveitides, including 156 cases of early-stage VKH and 103 cases of late-stage VKH, were evaluated. Overall accuracy for panuveitides was 96.3% in the training set and 94.0% in the validation set (95% confidence interval 89.0, 96.8). Key criteria for early-stage VKH included the following: (1) exudative retinal detachment with characteristic appearance on fluorescein angiogram or optical coherence tomography or (2) panuveitis with ≥2 of 5 neurologic symptoms/signs. Key criteria for late-stage VKH included history of early-stage VKH and either (1) sunset glow fundus or (2) uveitis and ≥1 of 3 cutaneous signs. The misclassification rates in the learning and validation sets for early-stage VKH were 8.0% and 7.7%, respectively, and for late-stage VKH 1.0% and 12%, respectively.

Conclusions: The criteria for VKH had a reasonably low misclassification rate and seemed to perform sufficiently well for use in clinical and translational research.

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Conflict of interest statement

Conflict of Interest: Douglas A. Jabs: none; Alastair K. Denniston: none; Andrew D. Dick: consultant: AbbVie, Alimera, Apitope, Astellas, Gyroscope, Janssen, Roche; James P. Dunn: none; Michal Kramer: none; Neal Oden: none; Peter McCluskey: none; Annabelle Okada: consultant: AbbVie Japan, Astellas Pharma Japan, Bayer AG, Daiichi Sankyo; lecture fees: Alcon Pharm Japan, Mitsubishi Tanabe Pharma, Novartis Pharma Japan, Santen Pharmaceutical Corporation, Senju Pharmaceutical Corporation; grant support from Alcon Pharma Japan, Bayer Yakuhin, Mitsubishi Tanabe Pharma; Alan G. Palestine: none; Russell Read: none; Jennifer E. Thorne: Dr. Thorne engaged in part of this research as a consultant and was compensated for the consulting service; Brett E. Trusko: none.

Figures

FIGURE 1.
FIGURE 1.
Serous retinal detachments in a patient with early-stage Vogt-Koyanagi-Harada disease. A. Color fundus photograph. B. Fluorescein angiogram, demonstrating multiloculated appearance.
FIGURE 2.
FIGURE 2.
Optical coherence tomogram of an exudative retinal detachment in a patient with early-stage Vogt-Koyanagi-Harada disease, demonstrating septate appearance.
FIGURE 3.
FIGURE 3.
Sunset glow fundus in a patient with late-stage Vogt-Koyanagi-Harada disease.

Comment in

References

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