[Vogt-Koyanagi-Harada syndrome: importance of rapid diagnosis and therapeutic intervention]

Abstract

Aim: The Vogt-Koyanagi-Harada (VKH) syndrome is characterized by a bilateral granulomatous uveitis with exudative retinal detachments associated with systemic manifestations such as meningeal signs, cutaneous signs (poliosis, alopecia and vitiligo) and dysacousis. VKH is relatively unfrequent in Europe and Switzerland. Therefore diagnosis is often reached with some delay. Our aim here was to analyze the 3 patients for whom the diagnosis was reached less than 15 days after the first signs and compare their evolution to seven patients for whom diagnosis was known one month or more after the first signs.

Patients and methods: Retrospective and partially prospective study of patients seen at uveitis clinic in Lausanne from 1990 to 1999 for whom an ICG angiographic work-up had been performed in addition to the usual clinical and fluoresceinic work-up. The frequency of VKH in our collective was calculated; symptoms and signs, paraclinical investigations, laboratory work-up, delay from first signs to diagnosis, the management and the evolution were the criteria analyzed. In particular the patients with early diagnosis and early treatment were analyzed and compared to the rest of the collective. Diagnosis was based on the criteria of the American Uveitis Society. Between 1990 and 1999, 14 patients with the diagnosis of VKH were seen (1.2% of our collective of uveitis patients). The 10 patients having had a work-up including ICG angiography in addition to the classical work-up were included in this study.

Results: The diagnosis was reached in less than 2 weeks in 3 patients. In all 3 patients inflammation was controlled after treatment. Two patients with a follow-up without recurrence of respectively 36 and 54 months were considered as healed. The last case had no recurrence after nine months but still was under therapy. Whereas clinical examination and fluorescein angiography failed to show any sequels in the 2 "healed" patients, ICG angiography showed numerous zones of hypofluorescence indicating choroidal scarring. For the 7 other cases, the diagnosis was reached one month or more after the first symptoms or signs and they all evolved in the chronic recurrent fashion. ICG angiography contributed to the rapid diagnosis in 2/3 patients with early diagnosis and was an essential parameter for the choroidal follow-up in 9/10 patients.

Conclusion: This study shows that it is essential to rapidly reach the diagnosis of VKH and treat the patients vigorously without delay. By showing choroidal lesions not seen by the clinical examination or fluorescein angiography. ICG angiography is essential for a correct work-up and follow-up of choroidal lesions in VKH. In our two "healed" patients it was the only mean to show choroidal sequellae.