IgG4相关疾病的临床病理学特征

Clinicopathological diagnosis of IgG4-related disease：report of eight cases

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。

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 （ ＆gt;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 ＆gt;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 .