전신성 홍반성 루푸스에서 병발한 시신경척수염 1예
A Case of Coexisting Neuromyelitis Optica in Systemic Lupus Erythematosus
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목적: 전신성 홍반성 루푸스를 가진 환자에서 병발한 시신경척수염을 경험하여 보고하고자 한다. 증례요약: 위궤양 및 대상포진 병력이 있는 26세 여자 환자가 좌안의 시력 저하로 본원에 입원하였다. 시신경염 의심 하에, 스테로이드치료 중 전신성 홍반성 루푸스로 진단되었고 치료 후 시력이 호전되었다. 11개월 후 하지로 진행하는 복부 및 좌측 옆구리 감각이상으로 입원하였다. 척수 자기공명영상에서 횡단척수염 소견이 보여 다발성 경화증을 의심하였다. 15개월 뒤 우안의 시력 저하 및 안통으로 입원하였고 스테로이드 치료로 호전되었다. 그러나 5주만에 우안 시신경염이 재발하였고 시신경척수염이 의심되어 azathioprine으로 치료하였다. Aquaporin-4 특이 자가항체(anti-aquaporin-4 Ab, NMO-IgG) 검사를 하고 혈청에서 양성 결과가 나왔다. 3개월만에하반신 마비로 입원하였고 척수 자기공명영상에서 종단으로 확장된 횡단척수염을 보여 시신경척수염이 확진되었고 고용량 스테로이드, 혈장교환술 및 rituximab으로 호전되었다. 이후 우안 시신경염이 재발하여 고용량 스테로이드 치료로 호전된 후 척수염이 재발하여고용량 스테로이드 및 혈장교환술로 호전되었다. 결론: 전신성 홍반성 루푸스와 같은 자가면역질환 환자에서 시신경염이 발생하였을 때 뇌병변 없이 횡단척수염 소견이 있으면서 자주재발하는 경우 시신경척수염의 병발을 반드시 고려해야 한다.
Abstract
Purpose: We present a case of a patient with coexisting neuromyelitis optica and systemic lupus erythematosus (SLE). Case summary: A 26-year-old female was hospitalized in our medical center due to decreased visual acuity in her left eye;she had a history of gastric ulcers and herpes zoster infection. Steroid treatment was started under suspicion of optic neuritis,and she was diagnosed with SLE. After treatment, her vision improved, but eleven months later she was hospitalized with paresthesia on the abdomen and left flank progressing to the lower extremities. Spinal MRI showed transverse myelitis,suggesting multiple sclerosis. Fifteen months later, the patient was hospitalized due to decreased visual acuity and ocular pain in the right eye. Her vision was improved by steroid therapy. However, optic neuritis recurred in the right eye after five weeks, thus azathioprine was added to the treatment. Anti-aquaporin-4 Ab test was conducted based on the suspicion of neuromyelitis optica, and the serum was positive for anti-aquaporin-4 Ab (NMO-IgG). The patient was hospitalized again due to paraplegia after three months. Coexistence of neuromyelitis optica was verified because spinal MRI showed longitudinally extensive transverse myelitis. The symptoms were improved by high doses of steroids, a series of plasmaphereses,and rituximab. Optic neuritis was repeated in the right eye and the symptoms were improved with high doses of steroids. Myelitis recurred later and the symptoms improved with high doses of steroids and a series of plasmaphereses. Conclusions: Coexisting neuromyelitis optica should be considered in cases with relapsing events which have transverse myelitis without cranial lesions in autoimmune diseases such as SLE.
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