스테로이드 무반응 시신경척수염 환자에서 혈장분리교환술의 효과
Efficacy of Plasma Exchange Therapy for Steroid-Unresponsive Neuromyelitis Optica
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초록
목적: 스테로이드 치료에 반응이 없었던 시신경척수염 환자에서 재발성 시신경염의 경우 시행한 혈장분리교환술의 치료효과를 알아보고자 하였다. 증례요약: 한쪽 눈 시신경염으로 스테로이드 치료를 시행하였으나, 증상의 호전이 없었던 세 명의 여자환자가 반대쪽에 발생한 급격한시력저하와 안운동 시 동반되는 안통으로 내원하였다. 시신경척수염 항체검사 양성, 안와 자기공명영상에서 시신경 조영증가를 보여시신경척수염으로 진단하였고, 스테로이드 치료를 시행하였으나 반응을 보이지 않아, 혈장분리교환술을 시행하였다. 혈장분리교환술직후 빠른 시력회복 및 시야의 호전을 보였고, 면역억제제로 유지요법을 시행한 후 시신경척수염의 재발을 보이지 않았다. 결론: 스테로이드 치료에 반응이 없는 시신경척수염 환자에서 즉각적인 혈장분리교환술시행을 고려해야 할 것으로 생각한다.
Abstract
Purpose: To report the therapeutic efficacy of plasma exchange therapy on steroid-unresponsive neuromyelitisoptica(NMO) patients. Case summary: Three patients who had not achieved improvement of visual acuity and visual field after high steroid pulsetherapy after optic neuritis visited our clinic with decreasing visual acuity combined with eye pain in the other eye. All patientswere diagnosed as neuromyelitisoptica (NMO) based on the presence of NMO-IgG antibody and optic nerve enhancingin contrast-enhanced orbital magnetic resonance imaging (MRI). Recurrent optic neuritis was observed. Steroidpulse retreatment was started but visual acuity was not improved in all patients. The patients received plasma exchangetherapy, followed by immune suppression therapy. All patients showed improved visual acuity and restored visual fieldpromptly without recurrence of neuromyelitisoptica. Conclusions: In case of steroid-unresponsive neuromyelitisoptica, plasma exchange should be promptly considered asthe treatment of choice.
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