핵주변항호중구세포질항체와 연관된 경수막염에 동반된 양안 안와첨부증후군
Orbital Apex Syndrome with Perinuclear Anti-neutrophil Cytoplasmic Antibody-associated Pachymeningitis
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목적: 핵주변항호중구세포질항체와 연관된 혈관염에 동반된 경수막염에서 발생한 양안 안와첨부증후군 환자 1예를 보고하고자 한다. 증례요약: 79세 여자가 1주 전에 발생한 두통과 양안 시력저하 및 눈운동장애를 주된 소견으로 내원하였다. 안와 자기공명영상검사상 특징적인 경막의 비후가 확인되고 비후된 경막이 시신경관과 상안와열에서 시신경과 눈운동신경을 압박하는 안와첨부증후군 소견이 관찰되었다. 혈액검사 결과 핵주변항호중구세포질항체가 확인되어 핵주변항호중구세포질항체 연관 경수막염으로 진단하였다. 고용량스테로이드 정주 및 사이클로포스파마이드 병행 치료를 하였으며, 이후 경구 아자티오프린 및 프레드니솔론을 유지하였다. 치료 후 핵주변항호중구세포질항체 역가가 음전되었고 눈운동장애는 모두 호전되었으나 시력은 전혀 호전되지 않았다. 결론: 핵주변항호중구세포질항체 연관 혈관염과 관련된 경수막염 환자에서 안와첨부증후군이 초기 증상으로 나타날 수 있으므로 감별하는 것이 중요하다.
Abstract
Purpose: To report a rare case of perinuclear anti-neutrophil cytoplasmic antibody-associated pachymeningitis presenting as bilateral orbital apex syndrome. Case summary: A 79-year-old female presented with a visual acuity decrease and ophthalmoplegia in both eyes from 1 week prior to her visit. Orbital magnetic resonance imaging showed diffuse hypertrophy and enhancement of the dura mater near the optic canal and superior orbital fissure causing orbital apex syndrome. The serum titer of perinuclear anti-neutrophil cytoplasmic antibody was elevated. Based on these findings, she was diagnosed with pachymeningitis related to perinuclear anti-neutrophil cytoplasmic antibody-associated vasculitis. The antibody titers decreased and ophthalmoplegia recovered after treatment with high dose intravenous steroid injection, cyclophosphamide, and long-term maintenance therapy with oral azathioprine and prednisolone. However, her visual acuity did not improve in either eye. Conclusions: Perinuclear anti-neutrophil cytoplasmic antibody associated vasculitis can present as orbital apex syndrome and should be considered as a differential diagnosis.
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