눈부속기를 침범한 Hyper-IgG4 증후군 3예
Three Cases of Hyper-IgG4 Syndrome Involving Ocular Adnexa
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목적: 눈부속기를 침범한 Hyper-IgG4 증후군 3예를 경험하였기에 보고하고자 한다. 증례요약: 66세 여자 환자가 1년 전부터 시작된 양안 윗눈꺼풀 부종으로 내원하였다. 안와 전산화단층촬영에서 양쪽 눈물샘 비대를 보여 좌측 눈물샘 절개생검을 시행하였고, 면역화학염색에서 IgG4 양성을 보이는 림프형질세포의 침윤 소견을 보였다. 내과에 의뢰하여 시행한 폐 및 신장조직검사에서도 같은 소견을 보여 눈물샘, 폐, 신장을 침범한 Hyper-IgG4 증후군으로 진단하였고, 스테로이드제 및 면역억제제 복용하며 경과관찰 중이다. 49세 여자 환자가 4년 전부터 시작된 좌안 윗눈꺼풀 종괴로 내원하여 절제생검을 시행하였고 51세 여자 환자가 5개월 전에 시작된 양안 눈물샘 비대로 내원하여 절개생검을 시행하였다. 조직검사상 IgG4 양성을 보이는 림프형질세포의 침윤 소견을 보여 눈부속기의 Hyper-IgG4 증후군으로 진단하였고, 내과에 의뢰하여 시행한 전신 검사에서 다른 장기의 침범소견은 없었다. 결론: 전신 자가면역질환으로 알려진 Hyper-IgG4 증후군에서 눈부속기 침범이 가능하며, 눈부속기에서 Hyper-IgG4 증후군 발생 시 전신 질환을 의심해 보아야 한다.
Abstract
Purpose: To report 3 cases of hyper-IgG4 syndrome involving ocular adnexa. Case summary: A 66-year-old woman presented with swelling of the bilateral upper eyelids with ocular pain that began 1 year before. Nodular episcleral injection of the left eye and other generalized symptoms, such as cough, decreased hearing ability, multiple nodular lesions of the bilateral lungs and right kidney, together suggested rheumatic disease. Orbital computed tomographic images revealed diffuse swelling of the bilateral lacrimal glands. After immunostaining a surgically-biopsied specimen from the lacrimal gland for IgG4 expression, 15% of infiltrated lymphoplasmacytic cells were IgG4-positive. Similar findings were shown with biopsied specimens from the lung and kidney; therefore, the patient was diagnosed with Hyper-IgG4 syndrome. A 49-year-old woman complained of a mass in the left upper eyelid that began 4 years earlier. Orbital computed tomographic images showed a 5-mm-sized mass in the left upper eyelid. Ocular adnexal Hyper-IgG4 syndrome was confirmed by the immunostained biopsy from the left upper eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. A 51-year-old woman presented with swelling of the bilateral lacrimal glands. Enlargement of the bilateral lacrimal glands were apparent in orbital computed tomographic images. After anti-IgG4 antibody staining of a biopsied specimen from the right lacrimal gland, dense infiltration of IgG4-positive lymphoplasmacytic cells was observed. The patient was also diagnosed with Hyper-IgG4 syndrome.
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