摘要
目的探讨IgG4相关性胆胰疾病呈局限性胰腺或胆管病变时与胰腺癌、胆管癌的鉴别要点及治疗方法。方法回顾性分析以黄疸、腹痛为主要症状就诊，并最终明确诊断的IgG4相关胰胆管疾病18例患者临床资料，其中Ⅰ型自身免疫性胰腺炎（I—AIP）7例，IgG4相关性硬化性胆管炎（IgG4-SC）4例，Ⅰ—AIP合并IgG4-SC 7例。结果（1）影像学：Ⅰ-AIP表现为胰头局灶肿块，增强扫描表现为相对乏血供肿块，延迟像呈较均匀强化，强化幅度超过正常胰腺；IgG4-SC受累胆管壁呈对称性环周狭窄和（或）腔内软组织团块，胆管狭窄区与非狭窄区域均可见胆管壁增厚，增厚胆管壁及腔内软组织团块增强扫描可见强化。（2）血清学：血清IgG4为59．3～1120．0mg／dl，中位值392．0（218．0．594．0）mg／dl；CA19—9为12．2～230．8U／ml。（3）组织病理学：主要表现为大片状浆细胞和淋巴细胞浸润及纤维化，部分免疫组织化学染色示IgG4阳性细胞〉10个／高倍视野，可见席纹状纤维化．个别可同时伴闭塞性静脉炎。（4）器官受累：合并其他器官病变者6例，其中肾损害、腹膜后纤维化2例．大网膜及肠系膜炎性结节各1例，颌下腺肿大2例，垂体炎1例。（5）对激素治疗的反应：6例手术患者中，2例病变手术后未予激素治疗，余4例患者病变未清除或伴其他脏器损害给予激素治疗：12例非手术患者中，2例患者自动出院，使用激素治疗者10例。治疗后影像学结果均有明显改善。结论以局限性胰腺或胆管病变为特征的IgG4相关性胆胰疾病，可通过影像学、血清IgG4升高、组织病理学、其他受累器官及激素诊断性治疗与胰腺癌或胆管癌相鉴别。
Objective To investigate the differential diagnosis and management of IgG4 associated biliary pancreatic diseases. Methods A total of 18 patients with jaundice and abdominal pain were retrospectively studied. The final diagnosis was 7 cases of type I autoimmune pancreatitis （ Ⅰ -AIP） , 4 cases of IgG4-associated sclerosing cholangitis （IgG4-SC） , and 7 cases of Ⅰ-AIP combined IgG4-SC. Results （1）Imaging features： Ⅰ-AIP patients showed pancreatic head mass, with relatively poor blood supply when under dynamic contrast-enhanced scan, in delay image, enhanced amplitude more than the normal pancreas. IgG4-SC can be seen in patients with symmetrical bile duct wall stenosis and （or） soft tissue mass, the thickened bile duct wall and intraluminal soft tissue mass can be enhanced with bile duct wall thickening. （2）Serological characterlstlcs：Serum IgG4 fluctuated between 59.3 and 1 120. 0 mg/dl, CA19-9 fluctuated between 12. 2 and 230. 8 U/ml. （3）Histopathology： Plasma cells lymphocyte infiltration and fibrosis, immunohistochemical staining showed IgG4 positive cells 〉 10/hpf, and obliterative phlebitis. （4）Combined with other organ lesions in 6 cases including renal injury, retroperitoneal fibrosis,mesenteric inflammatory nodules, omenta] inflammatory nodules, submandibular gland enlargementin, and pituitary inflammation . （5） Response to hormone therapy： 4 out of 6 operated patients were none resected and were given hormonotherapy . In twelve non-surgical patients,2 cases abandoned therapy, 10 cases were treated with hormone therapy, and improved. Conclusion IgG4 associated biliary pancreatic disease characterized by localized pancreatic or bile duct disease, can be distinguished from malignancy by special imaging, IgG4 elevation, histopathology and positive response to experimental hormonotherapy.
出处
《中华普通外科杂志》
CSCD
北大核心
2017年第2期149-152,共4页
Chinese Journal of General Surgery
关键词
自身免疫疾病
胰腺肿瘤
胆管肿瘤
Autoimmune diseases
Pancreatic neoplasms
Bile duct neoplasms
