안구운동장애를 동반한 호산구 변종의 육아종증다발혈관염 증례 1예
An Eosinophilic Variant of Orbital Granulomatosis with Polyangiitis with Ocular Motility Disorder
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목적: 안구운동장애를 동반하고 호산구 및 immunoglobulin E (IgE)의 증가를 초래하는, 안와에 국한된 육아종증다발혈관염(granulomatosis with polyangiitis, GPA)의 호산구 변종(eosinophilic variant) 증례를 보고한다. 증례요약: 특이 병력 없는 61세 남자로, 2일 전 발생한 양안 복시로 내원하였다. 최대교정시력 양안 1.0, 좌안의 눈꺼풀부종과 상방 및하방주시와 외전시 운동 제한을 보였다. 혈액검사에서 세포질 내 항중성구세포질항체 양성, 증가된 호산구 분율(28.3%, 2,462/mm3) 과 IgE (400 KU/L)가 이상 소견으로 나타났고, 자기공명영상에서 안와 하이측에 종괴, 눈물샘 확장과 외안근 주위 염증이 관찰되었다. 눈물샘 조직검사에서 국소적 호산구 침윤을 동반한 GPA의 변종으로 진단되었다. 안와 조직에 일차적으로 생성된 GPA에서 전신적호산구 및 IgE 증가증을 동반하는 호산구 변종으로 진단하고, 수술 없이 경구용 스테로이드 치료로 안구운동장애를 완치하였다. 결론: 급성 복시를 동반한 안와 염증 환자에서 드물게 GPA가 원인 질환일 수 있으므로 영상검사와 혈액검사를 통한 배제진단이 필요하다.
Abstract
Purpose: To present the case of a patient with localized orbital granulomatosis with polyangiitis (GPA) characterized by a significant ocular motility disorder, increased eosinophilia, and hyper-immunoglobulin E (hyper-IgE). Case Summary: A 61-year-old male, with no previous history of allergy, atopy, asthma, sinonasal disease, respiratory disease, or renal disease, came to our hospital due to a 2-day history of binocular diplopia. His left eye exhibited inward and upward deviation, along with gaze limitation. His vision was 20/20 in both eyes, and the pupillary light reflex and color test were normal. He had severe eyelid swelling and conjunctival injection without tenderness in his left eye, and retinal vessel congestion around the optic nerve, without proptosis. Laboratory tests revealed a positive cytoplasmic antineutrophil cytoplasmic antibody (cANCA), a normal WBC count, elevated eosinophils at 28.3% (2,462/mm3), and high serum levels of IgE (400 KU/L). Magnetic resonance imaging showed an inferolateral orbital mass with an enlarged lacrimal gland and myositis of the extraocular muscles. A biopsy of the lacrimal gland revealed nonspecific chronic inflammation with an eosinophilic infiltrate. The presence of cANCA, in combination with clinical and pathological findings, led to the diagnosis of an eosinophilic variant of localized orbital GPA. This variant was primarily confined to the orbital tissue, marked by elevated eosinophil and IgE levels, and was treated with oral steroids without requiring surgery for the ocular motility disorder. Conclusions: GPA may present as acute strabismus with orbital inflammation, even in the absence of systemic signs. Therefore, it should be considered in the differential diagnosis of unexplained acute orbital syndromes.
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