시신경을 침범한 IgG4 연관 경화성 질환 1예
A Case of IgG4-Related Sclerosing Disease Involving the Optic Nerve
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목적: 시신경을 침범한 IgG4 연관 경화성 질환 1예를 경험하였기에 보고하고자 한다. 증례요약: 59세 남자환자가 6년 전부터 시작된 시력저하 및 좌안 돌출을 주소로 내원하였다. 환자는 10여 년 전 림프종 의심하에 타병원에서 항암 치료를 받았으나 이후 자의적으로 치료를 중단하였으며 이후 안와 주위 부종 완화 위해 임의로 스테로이드를 복용하여왔다. 안와 자기공명영상에서 양측 시신경 및 외안근을 침범하는 미만성의 침윤성 병변이 관찰되었다. 좌측 눈물샘 후면과 외직근외측 사이에서 절개생검을 시행하였고, 면역화학염색에서 IgG4 양성을 보이는 림프형질세포의 침윤 소견을 보여, IgG4 연관 경화성질환 진단하에 고용량 스테로이드 치료를 시행하였다.
Abstract
Purpose: To report a case of IgG4-related sclerosing dacryoadenitis masquerading for a long period as a. Case summary: A 59-year-old man with visual acuity loss and proptosis in the left eye was referred to our hospital. Ten years prior, the patient was diagnosed with lymphoma and underwent chemotherapy at another hospital. However, the patient spontaneously stopped treatment and took steroids for the relief of periorbital swelling. Magnetic resonance imaging revealed diffuse infiltrative lesions involving both optic nerves and extraocular muscles. Incisional biopsies of the lacrimal gland’s posterior side and the lateral rectus muscle’s lateral side were performed. Staining for IgG4 via immunochemistry showed infiltration of IgG4-positive lymphoplasmacytic cells. Under the diagnosis of IgG4-related sclerosing disease, the patient underwent high-dose steroid pulse therapy.
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