Vogt-Koyanagi-Harada syndrome: Experience in a Belgian university hospital and review of the literature

Abstract

Introduction: The Vogt-Koyanagi-Harada syndrome (VKHS) is rare in Europe. Bilateral panuveitis is the defining characteristic, but the disease can affect other tissues containing melanin, such as the inner ear, the meninges, and the skin. Therefore, patients may present not only to ophthalmologists, but also to internists, dermatologists, ENT-physicians and neurologists. Early and aggressive immunosuppressive treatment is necessary in order to prevent permanent visual loss or systemic complications.

Objective: We retrospectively studied 12 patients diagnosed and treated in a specialized uveitis clinic in Leuven (Belgium) between 2005 and 2013, and we compared our data with literature.

Results:  The most common extra-ocular manifestations were neurological, with headache (100%) and lymphocytic meningitis (75%) as main findings. Tinnitus was present in 50%, and vertigo in 42%. Vitiligo was the most frequent dermatological manifestation and present in 42%. Uveitis recurred in 67% of patients with 1 to 4 relapses per patient and a median time to recurrence of 6 months (range 1-11). Normal visual acuity was seen in 75% of patients at the end of follow-up (5 to 60 months), but 3 patients had at least some degree of permanent visual impairment in at least one eye.

Conclusion: VKHS is a rare, multisystem disorder, characterized by bilateral uveitis and variable neurologic, auditory and skin symptoms. A lumbar puncture increases the diagnostic yield. A fast diagnosis allows timely initiation of adequate therapy, and If treated correctly, VKHS has a favorable prognosis. It is unclear if initial intravenous therapy improves frequency and time to remission.