Horiuchi Katsuhiko, Kakizaki Satoru, Kosone Takashi, Ichikawa Takeshi, Sato Ken, Takagi Hitoshi, Mori Masatomo, Sakurai Shinji, Fukusato Toshio
Department of Gastroenterology, Kusunoki Hospital, Fujioka.
Intern Med. 2009;48(16):1377-82. doi: 10.2169/internalmedicine.48.2223. Epub 2009 Aug 17.
We encountered a 45-year-old man who presented with marked eosinophilia as the first manifestation of sclerosing cholangitis. He was found to have a liver dysfunction during a regular physical check up and thereafter consulted our hospital. The laboratory data on admission indicated an elevation of AST (96 IU/L), ALT (136 IU/L) and ALP (1,025 IU/L). Furthermore, the leukocyte count was 18,190/mm(3) and he also showed marked eosinophilia (54.5%, 9,914/mm(3)). There were no atypical findings in the eosinophils. Other diseases causing eosinophilia, including parasite infection, allergic disorders, hypereosinophilic syndromes, drug-induced eosinophilia, malignancies, etc. were all investigated and ruled out. A liver biopsy revealed marked eosinophilic infiltration in the portal area and interlobular bile duct injury. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a slight dilatation of the left intrahepatic bile ducts, but no clear diagnosis could be made at that time. A follow-up liver biopsy and endoscopic retrograde cholangiopancreatography (ERCP) finally revealed a diagnosis of secondary sclerosing cholangitis due to eosinophilic cholangiopathy. According to previous Japanese reports, eosinophilia of more than 5% was reported in 39 of 142 (27.0%) primary sclerosing cholangitis (PSC) patients. Eosinophilic cholangiopathy could cause a condition mimicking PSC and it might be confused as PSC with eosinophilia. The literature contains only about 40 case reports on eosinophilic cholangiopathy, and therefore, to date little attention has been paid to this condition. We should therefore pay attention to this condition when making a differential diagnosis of either PSC or IgG4-related sclerosing cholangitis.
我们遇到一名45岁男性，其以明显嗜酸性粒细胞增多作为硬化性胆管炎的首发表现。他在常规体检时发现肝功能异常，随后前来我院就诊。入院时实验室检查数据显示，谷草转氨酶（AST，96 IU/L）、谷丙转氨酶（ALT，136 IU/L）和碱性磷酸酶（ALP，1025 IU/L）升高。此外，白细胞计数为18,190/mm³，且他还表现出明显的嗜酸性粒细胞增多（54.5%，9,914/mm³）。嗜酸性粒细胞未见非典型表现。对包括寄生虫感染、过敏性疾病、高嗜酸性粒细胞综合征、药物性嗜酸性粒细胞增多、恶性肿瘤等在内的其他导致嗜酸性粒细胞增多的疾病均进行了排查并排除。肝脏活检显示门管区有明显嗜酸性粒细胞浸润及小叶间胆管损伤。磁共振胰胆管造影（MRCP）显示肝内左叶胆管轻度扩张，但当时无法明确诊断。后续肝脏活检及内镜逆行胰胆管造影（ERCP）最终确诊为嗜酸性胆管病继发硬化性胆管炎。根据既往日本的报告，142例原发性硬化性胆管炎（PSC）患者中有39例（27.0%）嗜酸性粒细胞增多超过5%。嗜酸性胆管病可导致类似PSC的情况，可能会与伴有嗜酸性粒细胞增多的PSC相混淆。关于嗜酸性胆管病的文献报道仅有约40例，因此，迄今为止该疾病很少受到关注。所以，在对PSC或IgG4相关硬化性胆管炎进行鉴别诊断时，我们应留意这种情况。
