Zhang Haibo, Liu Yaling, Li Zhenfei, Liang Na, Zhou Xiaomeng, Nie Xiangyu, Zhang Ting, Qi Weijing
Department of Neurology, The Second Hospital of Hebei Medical University, Shijiazhuang, China.
Key Laboratory of Neurology of Hebei Province, Shijiazhuang, China.
Front Neurol. 2019 Aug 14;10:890. doi: 10.3389/fneur.2019.00890. eCollection 2019.
Amyotrophic lateral sclerosis (ALS) is a disease of which the underlying etiology and pathogenesis are unknown. Numerous data indicate an important role of the immune system and mitochondrial function in the disease. Primary biliary cirrhosis (PBC) is an autoimmune liver disease resulting from a combination of genetic and environmental risk factors. Patients with PBC develop innate and adaptive immune reactions against mitochondrial antigens. Therefore, common mechanisms could exist in both diseases. We present two cases of ALS with PBC to explore the relationship between the two diseases from the immunological and mitochondrial aspects. Further attention should be given to immune-modulating therapy in ALS patients.
肌萎缩侧索硬化症（ALS）是一种病因和发病机制尚不清楚的疾病。大量数据表明免疫系统和线粒体功能在该疾病中起重要作用。原发性胆汁性肝硬化（PBC）是一种由遗传和环境风险因素共同导致的自身免疫性肝病。PBC患者会针对线粒体抗原产生先天性和适应性免疫反应。因此，这两种疾病可能存在共同机制。我们报告两例合并PBC的ALS病例，从免疫和线粒体方面探讨这两种疾病之间的关系。对于ALS患者，应进一步关注免疫调节治疗。
