Department of Biomedical Sciences, Humanitas University, Pieve Emanuele 20090, Milan, Italy.
World J Gastroenterol. 2022 Apr 14;28(14):1430-1443. doi: 10.3748/wjg.v28.i14.1430.
Primary biliary cholangitis and primary sclerosing cholangitis (PSC) are the most common cholestatic liver diseases (CLD) in adults and are both characterized by an immune pathogenesis. While primary biliary cholangitis is a model autoimmune disease, with over 90% of patients presenting very specific autoantibodies against mitochondrial antigens, PSC is considered an immune mediated disease. Osteoporosis is the most common bone disease in CLD, resulting in frequent fractures and leading to significant morbidity. Further, sarcopenia is emerging as a frequent complication of chronic liver diseases with a significant prognostic impact and severe implications on the quality of life of patients. The mechanisms underlying osteoporosis and sarcopenia in CLD are still largely unknown and the association between these clinical conditions remains to be dissected. Although timely diagnosis, prevention, and management of osteosarcopenia are crucial to limit the consequences, there are no specific guidelines for management of osteoporosis and sarcopenia in patients with CLD. International guidelines recommend screening for bone disease at the time of diagnosis of CLD. However, the optimal monitoring strategies and treatments have not been defined yet and vary among centers. We herein aim to comprehensively outline the pathogenic mechanisms and clinical implications of osteosarcopenia in CLD, and to summarize expert recommendations for appropriate diagnostic and therapeutic approaches.
原发性胆汁性胆管炎和原发性硬化性胆管炎(PSC)是成人中最常见的胆汁淤积性肝病(CLD),两者均以免疫发病机制为特征。虽然原发性胆汁性胆管炎是一种典型的自身免疫性疾病,超过 90%的患者针对线粒体抗原存在非常特异性的自身抗体,但 PSC 被认为是一种免疫介导的疾病。骨质疏松症是 CLD 中最常见的骨骼疾病,导致频繁骨折,并导致显著的发病率。此外,肌肉减少症正成为慢性肝病的常见并发症,对患者的预后有显著影响,并严重影响其生活质量。CLD 中骨质疏松症和肌肉减少症的发病机制在很大程度上仍不清楚,这些临床情况之间的关联仍有待分析。尽管及时诊断、预防和管理骨质疏松症和肌肉减少症对于限制其后果至关重要,但目前尚无针对 CLD 患者骨质疏松症和肌肉减少症管理的具体指南。国际指南建议在 CLD 诊断时筛查骨骼疾病。然而,尚未确定最佳的监测策略和治疗方法,并且在各个中心之间存在差异。本文旨在全面概述 CLD 中骨质疏松症和肌肉减少症的发病机制和临床意义,并总结专家对适当诊断和治疗方法的建议。