This week I’m reading articles about VKH, a rare inflammatory eye disease that includes a prodrome of aseptic meningitis, inner ear involvement and sometimes other neurologic symptoms, as well as autoimmune skin changes like vitiligo and poliosis.
Study motivation: VKH is a heterogeneous disorder without biomarkers; clinical definitions exist but it’s hard to identify patients for research that have the disorder while accurately excluding those who don’t. This study tried to identify key clinical features that would allow for accurate diagnosis.
Study design: 10 uveitis centers prospectively collected data on every patient presenting with bilateral ocular inflammatory disease within a 3-month period for statistical analysis (total of 1147 patients, of whom 180 were diagnosed with VKH).
Findings: exudative retinal detachments were highly associated with acute VKH (PPV 100%, NPV 88%), while sunset glow was diagnostic of chronic VKH (PPV 94%, NPV 89%). Adding other common clinical features like vitiligo and alopecia didn’t improve the positive or negative predictive values.