CAJ | 학술논문

目的：探讨IgG4相关疾病（ IgG4RD）的临床和病理形态特点，提高对IgG4RD的认识和诊断水平。方法收集8例IgG4RD的临床和病理组织学资料，其中2例为胆总管及部分肝切除标本，2例为颌下腺切除标本，1例为肺穿刺标本，1例为开胸肺活检标本，1例为肾穿刺活检标本，1例为肾切除标本。切除标本均经充分取材，石蜡包埋，行 HE、弹力纤维组织化学及免疫组织化学（ MaxVision法）染色。结果患者男性5例、女性3例，平均年龄60岁。5例在术前都拟诊为肿瘤：2例拟诊为胆总管腺癌，2例拟诊为涎腺肿瘤，1例拟诊为肾盂癌。血清学检查：8例血清IgG4升高，5例IgE升高，4例嗜酸性粒细胞升高，4例为系统性病变。病理学特点：病灶内见大量淋巴细胞、浆细胞浸润，有淋巴滤泡形成，4例伴多量的嗜酸性粒细胞浸润；原组织结构破坏，伴有不同程度的编织状纤维化；IgG4阳性的浆细胞＞20～50个/HPF，IgG4/IgG＞40％；4例可见闭塞性静脉炎；未见坏死性血管炎、淋巴瘤等相应的病理改变。5例接受糖皮质激素治疗的患者反应良好，临床症状改善。结论 IgG4RD有相对较特异的病理形态改变，计算病灶中IgG4阳性的浆细胞绝对数量和相对数量，可获得明确诊断，但须结合临床除外其他可致IgG4升高的疾病。 IgG4临床表现各异，需及早明确诊断，糖皮质激素治疗有效。
목적：탐토IgG4상관질병（ IgG4RD）적림상화병리형태특점，제고대IgG4RD적인식화진단수평。방법수집8례IgG4RD적림상화병리조직학자료，기중2례위담총관급부분간절제표본，2례위합하선절제표본，1례위폐천자표본，1례위개흉폐활검표본，1례위신천자활검표본，1례위신절제표본。절제표본균경충분취재，석사포매，행 HE、탄력섬유조직화학급면역조직화학（ MaxVision법）염색。결과환자남성5례、녀성3례，평균년령60세。5례재술전도의진위종류：2례의진위담총관선암，2례의진위연선종류，1례의진위신우암。혈청학검사：8례혈청IgG4승고，5례IgE승고，4례기산성립세포승고，4례위계통성병변。병이학특점：병조내견대량림파세포、장세포침윤，유림파려포형성，4례반다량적기산성립세포침윤；원조직결구파배，반유불동정도적편직상섬유화；IgG4양성적장세포＞20～50개/HPF，IgG4/IgG＞40％；4례가견폐새성정맥염；미견배사성혈관염、림파류등상응적병리개변。5례접수당피질격소치료적환자반응량호，림상증상개선。결론 IgG4RD유상대교특이적병리형태개변，계산병조중IgG4양성적장세포절대수량화상대수량，가획득명학진단，단수결합림상제외기타가치IgG4승고적질병。 IgG4림상표현각이，수급조명학진단，당피질격소치료유효。
Objective To evaluate the clinical and pathological features of IgG 4-related disease (IgG4RD).Methods The clinical data, laboratory profiles, radiological, pathological and therapeutic features of eight cases of IgG 4RD were analyzed .This cohort included two cases of common bile duct and partial hepatectomy specimens , two of submandibular gland excision specimens , one from lung biopsy specimen , one from open lung biopsy specimen , one from renal biopsy specimen ,and one from renal excision specimen.In all cases,adequate lesion tissues were obtained.They were paraffin embedded , HE stained, and additional special stains and immunohistochemistry performed ( MaxVision method ).Results This series consisted of five males and three females , with a mean age of onset of 60 years.Five cases were suspected to be malignant pre-operatively, including two cases suspected of common bile duct carcinoma , two suspected of salivary gland tumor , and one suspected of renal pelvic carcinoma.Elevated serum levels of IgG4 and IgE were detected in five cases and eosinophilia in four cases.Multi-organ involvement was noted in four cases.The major histopathological features associated with IgG 4-RD were:dense lymphoplasmacytic infiltrate, with lymphoid follicle formation.Extensive eosinophilic infiltrate ( >10/HPF) was seen in four cases;fibrosis that was arranged at least focally in a storiform pattern was also noted.The numbers of IgG4 positive plasma cells were >20-50/HPF, while the IgG4 to IgG ratio was more than 40%.Obliterative phlebitis was present in four cases.Other pathological changes such as necrotizing vasculitis or lymphoma were not found.Five patients responded well to glucocorticoids.Conclusions IgG4RD has relatively specific histopathological features;accurate evaluation of the absolute and relative number of IgG 4 positive plasma cells in lesional tissue , combining with clinical examination and exclusion of other causes of elevated IgG4, allows the diagnosis of IgG4RD.IgG4RD has complicated clinical manifestation , and glucocorticoids therapy is efficacious .