IgG4相关硬化性胆管炎临床病理分析

The Clinicopathological Analysis of IgG4-related Sclerosing Cholangitis

目的探讨Ig G4相关硬化性胆管炎(Ig G4-SC))的临床病理学特点。方法回顾性分析10例Ig G4相关硬化性胆管炎的组织形态、免疫组织化学、影像特点,并对其治疗和预后进行分析。结果 10例Ig G4-SC患者均表现为血清Ig G4水平升高,胆管壁及腺体周围大量Ig G4阳性浆细胞浸润和组织纤维化、硬化,胆管周围炎症反应显著,胆管上皮细胞保持完整,间质内炎症细胞CD38,CD138阳性,Ig G4阳性细胞占Ig G阳性浆细胞的40%,胆管成像显示弥漫性或节段性胆管狭窄、扩张,胆管壁增厚。结论 Ig G4相关硬化性胆管炎有其特有的临床病理表现,短期内小剂量糖皮质激素治疗有效。

Objective To explore the clinicopathological characterristics of Ig G4-related sclerosing cholangitis. Methods Ten cases of patients with Ig G4-related sclerosing cholangitis were retrospectively analysed of morphology,immunohistochemistry,image features,and the treatments and prognosis were analyzed. Results All of 10 patients showed the elevated serum Ig G4 levels. Bile duct wall and gland were around a large number of Ig G4 positive plasma cells infiltration and tissue fibrosis and sclerosis,and the inflammatory reaction was significant around the bile duct,bile duct epithelial cells remained intact,the interstitial inflammatory cells CD38,CD138 were positive. Ig G4 positive cells accounted for 40% of Ig G positive plasma cells,bile duct imaging showed diffuse or segmental bile duct stenosis,dilation,bile duct wall thickening. Conclusion Ig G4-related sclerosing cholangitis has its specific clinical and pathological characteristics. And low-dose glucocorticoid therapy is effective in the short-term.