Classification Criteria for Vogt-Koyanagi-Harada Disease

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.