스테로이드 감량 치료 중 재발을 보인 보크트-고야나기-하라다증후군의 임상양상
Clinical Features of Recurred Vogt-Koyanagi-Harada Syndrome during Oral Steroids Tapering Therapy
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목적: 고용량 정맥 스테로이드 치료 후 경구 스테로이드로 감량 중 재발하는 보크트-고야나기-하라다증후군의 임상양상을 알아보고자 한다. 대상과 방법: 2010년 1월부터 2017년 12월까지 보크트-고야나기-하라다증후군으로 진단받아 치료받은 환자 17명, 33안을 대상으로 연속적 후향적 의무기록을 분석하였다. 스테로이드 감량 치료 중 포도막염이나 장액망막박리가 다시 발생한 경우를 재발이라 정의하고, 재발한 군과 비재발군의 치료 전, 치료 후 24주째의 최대교정시력과 최대 망막하액의 높이, 중심망막두께, 맥락막두께, 스테로이드 감량 치료 기간, 빛간섭단층촬영 등을 분석하였다. 결과: 5명(8안)에서 경구 스테로이드 감량 치료 중 사라졌던 망막하액이 다시 관찰되었으며 재발한 환자군은 경구 스테로이드 증량, 경구 면역억제제 복용, 테논낭하 트리암시놀론 주사를 상황에 맞게 병용하였다. 사라졌던 망막하액이 다시 관찰된 환자를 포함하여, 17명의 환자 모두 치료 24주 후 장액망막박리가 완전히 흡수되었으며, 최대교정시력은 두 군에서 유의한 차이를 보이지 않았다. 치료 전 최대 망막하액의 높이와 중심망막두께는 재발군에서 비재발군에 비해 통계적으로 유의하게 높게 측정되었다(p=0.04). 결론: 보크트-고야나기-하라다증후군에서 고용량 정맥 스테로이드 치료 후 경구 스테로이드 감량 시 사라졌던 장액망막박리가 재발할 수 있으며, 망막중심오목에서 치료 전 최대 망막하액의 높이와 중심망막두께는 재발과 관련있는 인자로 추측된다. 재발한 경우에도 상황에 맞는 적극적인 치료를 통해 좋은 시력예후를 기대할 수 있을 것으로 생각한다.
Abstract
Purpose: To evaluate the clinical features of recurrent Vogt-Koyanagi-Harada syndrome during steroid tapering after high-dose intravenous steroid therapy. Methods: We retrospectively reviewed the medical records of eight patients who were diagnosed and treated for Vogt-Koyanagi- Harada syndrome from January 2010 to December 2017. Recurrence was defined as the occurrence of uveitis or serous retinal detachment during oral steroid tapering. We grouped the patients into two groups according to recurrence, and compared the best-corrected visual acuity, maximum height of subretinal fluid, central subfield thickness, choroidal thickness, average steroid tapering period and images from optical coherence tomography. Results: Seventeen patients were included and five patients (29.4%) recurred during oral steroid tapering. Patients with recurrent episodes were treated with increasing doses of oral steroid and/or oral immunosuppressant and sub-tenon injection of triamcinolone. Serous retinal detachment was completely absorbed after 24 weeks of treatment in all 17 patients, including the recurrence group and the best-corrected visual acuity was not significantly different between the two groups. The heights of the subretinal fluid and thicknesses of the central subfield before treatment were significantly higher in the recurrence group than in the non-recurrence group. Conclusions: Serous retinal detachment can recur during oral steroid tapering after high-dose intravenous steroid treatment in patients with Vogt-Koyanagi-Harada syndrome suggesting that the height of the subretinal fluid at the fovea and central subfield thickness before treatment are factors associated with recurrence. Even in the case of recurrence, good visual prognosis can be expected after additional treatment according to the clinical situation.
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