背景:免疫球蛋白G4相关疾病(IgG4-RD)可累及全身各个器官,主要表现为内分泌功能障碍,视力障碍,黄疸,和有限的性功能。IgG4相关的自身免疫性胰腺炎是由自身免疫反应引发的,其特征是胰腺和胰管的结构变化。该病主要累及中老年男性,通常表现为进行性无痛性黄疸,误诊为胆管癌或胰腺癌。
方法:本研究报告一名54岁的男性因糖尿病多次咨询不同机构,胰腺炎,肝酶升高,和黄疸。
方法:磁共振成像显示胰腺头部肿胀,尾部萎缩性。肝脏和胰腺组织病理显示IgG4浆细胞浸润,而肝活检显示界面性肝炎,肝纤维化,和假条形成,没有胆管损伤的证据.
方法:激素治疗后,患者的血清IgG4水平和肝酶水平恢复正常。
结果:该疾病在维持激素治疗2年后复发,患者接受了额外的激素诱导缓解治疗联合硫唑嘌呤。
结论:本研究报告的目的是提高对IgG4-RD的认识和理解,强调考虑到其复发的个性化治疗策略的必要性,协会,和成像功能。本报告为临床医生管理和诊断IgG4-RD患者提供了有价值的见解和指导。
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) can involve various organs throughout the body, primarily manifesting as endocrine dysfunction, visual impairment, jaundice, and limited sexual function. IgG4-related autoimmune pancreatitis is triggered by autoimmune reactions and characterized by structural changes in the pancreas and pancreatic ducts. The disease mainly affects middle-aged and elderly males, typically presenting as progressive painless jaundice and misdiagnosed as cholangiocarcinoma or pancreatic cancer.
METHODS: This study
reports a 54-year-old male who consulted with different institutions multiple times due to diabetes, pancreatitis, elevated liver enzymes, and jaundice.
METHODS: Magnetic resonance imaging revealed swollen head of the pancreas and atrophic tail. Liver and pancreatic tissue pathology showed IgG4 plasma cell infiltration, while liver biopsy indicated interface hepatitis, liver fibrosis, and pseudolobule formation, with no evidence of bile duct damage.
METHODS: Following hormone therapy, the patient\'s serum IgG4 levels and liver enzyme levels returned to normal.
RESULTS: The disease relapsed 2 years after maintaining hormone therapy, and the patient underwent additional hormone-induced remission therapy combined with azathioprine.
CONCLUSIONS: The purpose of this research report is to enhance the awareness and understanding of IgG4-RD, emphasizing the necessity for personalized treatment strategies that take into account its recurrence, associations, and imaging features. This report provides valuable insights and guidance for clinicians in managing and diagnosing patients with IgG4-RD.