Sun Yu, Shao Chen, Qu Hao, Zuo Gang, Jing Tao, Wan Taihu, Ji Shangwei
Department of Interventional Radiology, China-Japan Union Hospital of Jilin University, Changchun, Jilin Department of Pathology, Beijing Youan Hopsital of Capital Medical University Peking Union Medical College Hospital, Beijing, China.
Medicine (Baltimore). 2018 Sep;97(37):e12395. doi: 10.1097/MD.0000000000012395.
Primary biliary cholangitis (PBC) is a liver autoimmune disease. If this disease is associated with other liver injury factors, both misdiagnosis and missed diagnosis will easily occur. Therefore, detailed disease history collection and related laboratory examination should be performed on patients with liver injury for unidentified causes. When necessary, liver biopsy should be performed to confirm the histopathological diagnosis.
The subject patient was a 63-year-old Chinese male with chronic liver injury who had a drinking history of about 30 years and drank 500 g daily on average and began to take health products and dietary supplements (multivitamins) since June 2014.
Drug-induced liver injury (DILI) and alcoholic fatty liver disease (AFLD) were initially considered because the patient had a history of using health products (HP) and dietary supplements (DS) and drinking alcohol. However, he was subsequently considered with PBC based on the findings of anti-mitochondrial antibody positivity and elevated immunoglobulin level. Obstructive jaundice and space-occupying lesion in the liver were excluded by imaging examinations. Liver biopsy was performed to confirm the reasons for liver injury. Histopathological examination was conducted, and the patient was diagnosed with PBC associated with DILI and alcoholic liver fibrosis.
Ursodeoxycholic acid, glycyrrhizic acid, and methylprednisolone (small dose) were used to treat the patient.
After 2 months, the serum levels of ALT, AST, AKP, GGT, and globulin returned to normal. After 4 months, the patient showed liver injury once again (an increase in ALT, AST, AKP, GGT and GLB) caused by repaglinide administration due to hyperglycemia. Ursodeoxycholic acid and methylprednisolone replaced the repaglinide administration. After 3 weeks, the levels of ALT, AST, AKP, GGT, and GLB returned to normal again.
The correct knowledge on PBC and early-stage recognition and diagnosis should be emphasized. When other causes of the liver injury cannot be excluded, liver biopsy is suggested. Histopathological change can be used to further clarify the reasons for liver injury and the principal contradiction as well as to guide the theraputic regimen.
原发性胆汁性胆管炎（PBC）是一种肝脏自身免疫性疾病。如果该疾病与其他肝损伤因素相关，很容易发生误诊和漏诊。因此，对于病因不明的肝损伤患者，应详细收集病史并进行相关实验室检查。必要时，应进行肝活检以确诊组织病理学诊断。
该患者为一名63岁的中国男性，有慢性肝损伤，有大约30年饮酒史，平均每天饮酒500克，自2014年6月起开始服用保健品和膳食补充剂（多种维生素）。
最初考虑药物性肝损伤（DILI）和酒精性脂肪肝病（AFLD），因为患者有使用保健品（HP）和膳食补充剂（DS）及饮酒史。然而，随后根据抗线粒体抗体阳性和免疫球蛋白水平升高的结果，考虑为PBC。通过影像学检查排除了梗阻性黄疸和肝脏占位性病变。进行肝活检以确定肝损伤的原因。进行了组织病理学检查，患者被诊断为与DILI和酒精性肝纤维化相关的PBC。
使用熊去氧胆酸、甘草酸和甲泼尼龙（小剂量）治疗该患者。
2个月后，谷丙转氨酶（ALT）、谷草转氨酶（AST）、碱性磷酸酶（AKP）、γ-谷氨酰转肽酶（GGT）和球蛋白的血清水平恢复正常。4个月后，患者因高血糖服用瑞格列奈后再次出现肝损伤（ALT、AST、AKP、GGT和球蛋白升高）。熊去氧胆酸和甲泼尼龙替代了瑞格列奈的使用。3周后，ALT、AST、AKP、GGT和球蛋白水平再次恢复正常。
应强调对PBC的正确认识以及早期识别和诊断。当不能排除其他肝损伤原因时，建议进行肝活检。组织病理学改变可用于进一步明确肝损伤的原因和主要矛盾，并指导治疗方案。
