Papapoulos S E
Department of Endocrinology & Metabolic Diseases, University Hospital, AA Leiden, The Netherlands.
Baillieres Clin Endocrinol Metab. 1997 Apr;11(1):117-43. doi: 10.1016/s0950-351x(97)80553-8.
Paget's disease of bone is a focal disorder of bone remodelling due to abnormally increased osteoclast-mediated bone resorption. It rarely presents before the age of 35 years and its prevalence increases with age affecting 2-5% of the population above 50 years, making Paget's disease the most common skeletal disorder after osteoporosis. Its aetiology is not known but available evidence favours an infection of genetically predisposed individuals with a paramyxovirus. Affected bones change in shape, size and direction causing considerable morbidity but the majority of patients are asymptomatic. In Paget's disease increased bone resorption is tightly linked to increased bone formation which are reflected in the proportional increases in biochemical indices of bone turnover. Because the primary abnormality lies in the osteoclasts, inhibitors of bone resorption are used for its therapy and bisphosphonates are currently the treatment of choice. These decrease bone turnover effectively and their effect lasts after stopping treatment. With available bisphosphonates clinical and biochemical, long-lasting, remissions can be obtained in the majority of patients. Symptomatic disease and preparation for orthopaedic surgery are no longer the only indication for treatment, but asymptomatic patients with localizations at sites likely to induce complications should be considered candidates for bisphosphonate therapy.
骨Paget病是一种由于破骨细胞介导的骨吸收异常增加而导致的局限性骨重塑疾病。该病很少在35岁之前出现，其患病率随年龄增长而增加，影响50岁以上人群的2% - 5%，使Paget病成为仅次于骨质疏松症的最常见骨骼疾病。其病因尚不清楚，但现有证据支持副粘病毒感染具有遗传易感性的个体。受累骨骼在形状、大小和方向上发生改变，导致相当大的发病率，但大多数患者无症状。在Paget病中，骨吸收增加与骨形成增加紧密相关，这反映在骨转换生化指标的相应增加上。由于主要异常在于破骨细胞，骨吸收抑制剂被用于其治疗，双膦酸盐目前是首选治疗药物。这些药物可有效降低骨转换，且停药后其作用仍持续存在。使用现有的双膦酸盐，大多数患者可获得临床和生化方面的长期缓解。有症状的疾病和骨科手术准备不再是治疗的唯一指征，但无症状且病变部位可能引发并发症的患者应被视为双膦酸盐治疗的候选者。
