PDF(Pubmed)
Abstract：
IgG4-related diseases are rare multisystem disorders involving salivary glands, retroperitoneum, pancreas, biliary tract, and liver. Isolated biliary strictures and gall bladder involvement are rare in such patients, and presentation with cholangitis and weight loss can misguide the diagnosis toward malignancy. Here, we report an interesting case of IgG4-related biliary stricture with gall bladder involvement, presented with cholangitis and weight loss. The initial symptoms and imaging were guided toward the malignant possibilities of cholangiocarcinoma and pancreatic carcinoma. However, endosonography, serology, and histopathology clinched the diagnosis of IgG4-related disease. The patient was managed without any biliary intervention and with antibiotics, steroids, and steroid-sparing agents. There was a relapse of disease during the steroids taper that improved after hiking its doses. The disease responded with medical management on follow-up. We demonstrated the effectiveness of steroid-sparing agents to treat IgG4-related diseases, especially to avert the steroid-related adverse effects. This case highlights the possible mislead for the diagnosis and delayed management of IgG4 disease due to shared clinical features with hepatobiliary malignancies and the effectiveness of noninvasive measures of management.
摘要：
IgG4相关疾病是涉及唾液腺的罕见多系统疾病，腹膜后,胰腺,胆道,还有肝脏.孤立的胆管狭窄和胆囊受累在这类患者中很少见，胆管炎和体重减轻的表现可能会误导诊断为恶性肿瘤。这里,我们报道了一个有趣的IgG4相关胆管狭窄伴胆囊受累的病例，表现为胆管炎和体重减轻。最初的症状和影像学检查被引导到胆管癌和胰腺癌的恶性可能性。然而,超声内镜,血清学,和组织病理学确定了IgG4相关疾病的诊断。患者在没有任何胆道干预和抗生素的情况下接受治疗，类固醇,和类固醇保护剂。类固醇锥度期间疾病复发，在增加剂量后有所改善。该疾病在随访中得到了医学治疗。我们证明了类固醇保护剂治疗IgG4相关疾病的有效性，尤其是避免类固醇相关的不良反应。由于与肝胆恶性肿瘤的共同临床特征和非侵入性管理措施的有效性，该病例突显了对IgG4疾病的诊断和延迟管理的可能误导。
