Fang Fang, Li Yanming, Sun Mingjun, Hu Songtao, Wang Zheng, Liu Dongge, Wang Chen
Department of Pathology, Beijing Hospital, Beijing 100730, China.
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Zhonghua Bing Li Xue Za Zhi. 2014 Sep;43(9):618-22.
To evaluate the clinical and pathological features of IgG4-related disease (IgG4RD).
The clinical data, laboratory profiles, radiological, pathological and therapeutic features of eight cases of IgG4RD 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).
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 IgG4-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.
IgG4RD has relatively specific histopathological features; accurate evaluation of the absolute and relative number of IgG4 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.
评估IgG4相关性疾病（IgG4RD）的临床和病理特征。
分析8例IgG4RD患者的临床资料、实验室检查结果、影像学、病理及治疗特征。该队列包括2例胆总管及部分肝切除标本、2例颌下腺切除标本、1例肺活检标本、1例开放性肺活检标本、1例肾活检标本和1例肾切除标本。所有病例均获取了足够的病变组织。将其进行石蜡包埋、苏木精-伊红（HE）染色，并进行额外的特殊染色和免疫组织化学检查（MaxVision法）。
该系列包括5例男性和3例女性，平均发病年龄为60岁。5例术前怀疑为恶性，包括2例怀疑胆总管癌、2例怀疑涎腺肿瘤和1例怀疑肾盂癌。5例检测到血清IgG4和IgE水平升高，4例有嗜酸性粒细胞增多。4例发现多器官受累。与IgG4-RD相关的主要组织病理学特征为：密集的淋巴细胞和浆细胞浸润，伴有淋巴滤泡形成。4例可见广泛的嗜酸性粒细胞浸润（>10/高倍视野）；还注意到至少局部呈席纹状排列的纤维化。IgG4阳性浆细胞数量>20-50/高倍视野，而IgG4与IgG的比例超过40%。4例存在闭塞性静脉炎。未发现其他病理变化，如坏死性血管炎或淋巴瘤。5例患者对糖皮质激素反应良好。
IgG4RD具有相对特异的组织病理学特征；准确评估病变组织中IgG4阳性浆细胞的绝对和相对数量，结合临床检查并排除IgG4升高的其他原因，可诊断IgG4RD。IgG4RD临床表现复杂，糖皮质激素治疗有效。
