CAJ | 학술논문

目的：探讨眼附属器IgG4相关性疾病（ IgG4－RD ）的临床病理学特征。方法收集2012至2013年眼附属器淋巴组织增生性疾病的病例103例，对其进行组织学观察、IgG和IgG4免疫组织化学EnVision法染色及血清学IgG和IgG4浓度检测，选取符合IgG4－RD诊断标准的病例17例，分析总结其临床和病理改变特点。结果（1）17例眼附属器IgG4－RD，男性9例，女性8例。年龄11～71岁，平均年龄48．5岁。17例均以眼睑肿胀或眼球突出就诊，11例可见双侧累及，6例为单侧累及。病程从5个月到7年，其中13例病程＞2年。6例既往有过敏性疾病（药物食物过敏）史。眼外常累及的部位有淋巴结、涎腺、肺脏和肾脏等。9例术后出现复发。5例口服激素有效，3例为部分有效。（2）血清学检查：17例IgG4浓度均＞1．35 g／L（1．49～14．88 g／L）。（3）组织学上，假性淋巴瘤样模式8例，混合型模式8例，硬化型1例。14例病变位于泪腺，3例病变累及眶内脂肪结缔组织，未见发生于结膜及眼睑的病例。17例中均可见到不同程度淋巴细胞和浆细胞浸润伴淋巴滤泡形成，程度不一的组织纤维化及嗜酸性粒细胞浸润，但未见典型的闭塞性静脉炎。（4）免疫组织化学染色：17例IgG4阳性浆细胞绝对值均＞50个／HPF， IgG4／IgG阳性浆细胞比值＞40％。结论眼附属器IgG4－RD在临床表现、实验室检查和组织学表现上都具有一定的临床病理学特征，应注意与该部位近似病变的鉴别诊断。
목적：탐토안부속기IgG4상관성질병（ IgG4－RD ）적림상병이학특정。방법수집2012지2013년안부속기림파조직증생성질병적병례103례，대기진행조직학관찰、IgG화IgG4면역조직화학EnVision법염색급혈청학IgG화IgG4농도검측，선취부합IgG4－RD진단표준적병례17례，분석총결기림상화병리개변특점。결과（1）17례안부속기IgG4－RD，남성9례，녀성8례。년령11～71세，평균년령48．5세。17례균이안검종창혹안구돌출취진，11례가견쌍측루급，6례위단측루급。병정종5개월도7년，기중13례병정＞2년。6례기왕유과민성질병（약물식물과민）사。안외상루급적부위유림파결、연선、폐장화신장등。9례술후출현복발。5례구복격소유효，3례위부분유효。（2）혈청학검사：17례IgG4농도균＞1．35 g／L（1．49～14．88 g／L）。（3）조직학상，가성림파류양모식8례，혼합형모식8례，경화형1례。14례병변위우루선，3례병변루급광내지방결체조직，미견발생우결막급안검적병례。17례중균가견도불동정도림파세포화장세포침윤반림파려포형성，정도불일적조직섬유화급기산성립세포침윤，단미견전형적폐새성정맥염。（4）면역조직화학염색：17례IgG4양성장세포절대치균＞50개／HPF， IgG4／IgG양성장세포비치＞40％。결론안부속기IgG4－RD재림상표현、실험실검사화조직학표현상도구유일정적림상병이학특정，응주의여해부위근사병변적감별진단。
Objective To study the clinicopathologic characteristics of IgG4-related disease in the orbital and periorbital tissue.Methods The clinical manifestations and pathologic features of 17 cases of IgG4-related disease affecting the orbital and periorbital tissue encountered during the period from 2012 to 2013 were studied.Results There were 9 male patients and 8 female patients.The age of patients ranged from 11 to 71 years ( mean =48.5 years ) .The main clinical manifestation was swelling of the eyelids:bilateral in 11 patients and unilateral in 6 patients.The duration of disease ranged from 5 months to 7 years (more than 2 years in 13 cases) .Six patients had history of allergic disorders.In addition to orbital/periorbital involvement, the disease also affected salivary gland, lymph node, lung and kidney.The disease relapsed in 9 patients.Amongst the 8 patients treated with steroids, 5 of them achieved complete remission and the remaining 3 patients had partial remission.The IgG4 level of the 17 cases ranged from 1.49 to 14.88 g/L.Histologic examination showed pseudolymphoma pattern in 8 cases, mixed pattern in 8 cases and sclerotic pattern in 1 case.There were various degrees of lymphoplasmacytic infiltrates ( with lymphoid follicle formation) and stromal fibrosis.Classical obliterative phlebitis was absent.Tissue eosinophilia was demonstrated in the 17 cases studied.Immunohistochemical study showed the presence of more than 50 IgG4-positive plasma cells per high-power field, with IgG4/IgG plasma cells ratio more than 40%. Conclusion There are characteristic clinical manifestations, pathologic features and laboratory findings in orbital and periorbital IgG4-related disease.Thorough understanding is important in arriving at a correct diagnosis.