Wang Kai-Chun, Liao Hsien-Tzung, Tsai Chang-Youh
Division of Allergy, Immunology and Rheumatology, Department of Medicine, Taipei Veterans General Hospital, Taipei, Taiwan.
Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan.
BMJ Case Rep. 2018 Jul 6;2018:bcr-2018-224814. doi: 10.1136/bcr-2018-224814.
An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only. Nevertheless, the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed. The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone.
一名85岁男性，表现为面色苍白及全身瘙痒性丘疹。实验室检查发现嗜酸性粒细胞增多、自身免疫性溶血性贫血、混合性高胆红素血症、胆汁淤积及血清IgG4水平升高。腹部超声和CT显示胆管进行性扩张，胰腺弥漫性肿大，胰头和胰体周围有细微的包膜样低密度边缘。内镜逆行胰胆管造影未发现结石相关的胆管梗阻，而内镜经乳头活检仅显示慢性炎症。尽管如此，仍推测诊断为IgG4相关疾病合并自身免疫性溶血性贫血。给予中等剂量甲泼尼龙后，临床症状和实验室异常情况有所改善。
