原发性胆汁性胆管炎继发骨质疏松的研究进展

Research progress of osteoporosis secondary to primary biliary cholangitis

原发性胆汁性胆管炎(primary biliary cholangitis,PBC)又称原发性胆汁性肝硬化,是一种以进行性、非化脓性肝内胆管炎为病理特征,最终发展为肝硬化的慢性自身免疫性肝病。骨质疏松症(osteoporosis,OP)是一种以骨密度下降、骨结构损伤和骨折风险增加为特征的全身性疾病。骨质疏松是PBC的常见并发症,随病情进展发病率逐渐增加,发病率和骨折风险均高于普通人群,正日益受到关注。骨质疏松的危险因素包括老年、女性、吸烟、过量饮酒、低体重、早绝经、类固醇激素治疗、低体力活动、维生素D和钙摄入减少等。PBC继发骨质疏松是由多种病理机制导致的,其中严重的骨吸收和缓慢的骨形成发挥了主要作用,遗传、性腺机能减退、脂溶性维生素缺乏、高脂血症等也参与骨质疏松发病。确诊PBC后应早期开始骨质疏松的预防和治疗。目前尚无治疗PBC并发骨质疏松患者的统一方案,可按照老年性骨质疏松和绝经后骨质疏松方案治疗,包括双膦酸盐、激素替代治疗、甲状旁腺激素、调脂药物、降钙素等。本文就PBC并发骨质疏松的临床表现、危险因素、病理机制及治疗方面的研究进展进行综述。

Primary biliary cholangitis（ PBC）,also called primary biliary cirrhosis,is a chronic autoimmune liver disease with the pathological characteristics of progressive and non-suppurative intrahepatic cholangitis,and ultimately cirrhosis. Osteoporosis（ OP）is a systematic disease characterized by a decrease in bone mass and a deterioration of the bone architecture,with an increased risk of fracture. Osteoporosis is a common complication of PBC,and the incidence increases gradually as the disease progresses. The morbidity and fracture risk are higher than the general population,which is receiving greater and greater attention. The risk factors for osteoporosis include older age,women,smoking,excessive drinking,low weight,early menopause,using steroid hormone,low physical activity and reduced intake of vitamin D and calcium. Osteoporosis secondary to PBC is caused by a variety of pathological mechanisms,in which severe bone resorption and slow bone formation play a major role. Besides,heredity,hypogonadism,fat-soluble vitamin deficiency and hyperlipidemia are also involved in the onset of osteoporosis. The prevention and treatment of osteoporosis should be started early after the diagnosis of PBC. There is no unified treatment for PBC patients with osteoporosis,and it can be treated according to the treatment criteria of senile osteoporosis and postmenopausal osteoporosis. The treatment options include bisphosphonates, hormone replacement therapy, parathyroid hormone, lipid-regulating drugs and calcitonin. This review discussed the research progress in clinical manifestation,risk factors,pathogenesis and therapy of PBC complicated with osteoporosis.