免疫球蛋白G4相关性硬化病的临床特点分析

Analysis of clinical characteristics on immunoglobulin G4-associated sclerosing disease

目的分析总结IgG4相关性硬化病的临床特点及诊治方案。方法回顾性分析2010—2011年本院收治的2例IgG4相关性硬化病患者的临床资料，结合文献报道，总结该疾病的临床特点及诊疗要点。结果2例患者术前诊断为胆管或胰腺恶性肿瘤，行胰十二指肠切除术后经病理检查确诊。本病发病率低，临扇i表现多样，影像学表现酷似恶性肿瘤，极易误诊，对激素治疗敏感，如能及时明确诊断则可避免不必要的手术。结论IgG4相关性硬化病临床表现酷似恶性肿瘤，了解其临床特点有助于诊断疾病及正确选择治疗方案。

Objective To summarize the clinical features and treatment protocols of immunoglobulin G4- associated sclerosing disease （ISD）. Methods By literature review, the clinical profiles of two patients with ISD who were admitted to our hospital between 2010 and 2011 were retrospectively analyzed, and the clinical characteristics and the keys of diagnosis and treatment were summarized. Results The two cases were preoperatively susceptible of having malignant bile duct or pancreatic neoplasms and pathologically confirmed as ISD after pancreatojujenectomy. Characterized by a low incidence and various clinical manifestations, ISD may imageologically mimic malignant tumors leading to misdiagnosis. Treatment with steroids appeared effective and timely diagnosis could help to avoid unnecessary surgery. Conclusion Summarizing the clinical characteristics of ISD, a disease mimicking malignant tumors, may be helpful to the diagnosis and decision-making of treatment protocols.