University of Texas McGovern Medical School, Houston, TX, USA.
University of Texas McGovern Medical School, Houton, TX, USA.
Am Fam Physician. 2020 Aug 15;102(4):224-228.
Paget disease of bone is a benign disorder characterized by focal areas of increased bone turnover in one or more skeletal sites. It usually affects older adults, and men are at a higher risk than women. Any bone may be affected, but the disease has a high preference for the pelvis, spine, skull, and long bones. Pain is the most common symptom, and presentation of the disease may depend on which bones are affected, the extent of involvement, and the presence of complications. Paget disease of bone may be asymptomatic, and suspicion arises from incidental findings of elevated serum alkaline phosphatase levels on routine blood work or abnormalities on imaging tests performed for an unrelated cause. Evidence-based guidelines recommend the use of plain radiography and serum alkaline phosphatase testing for initial diagnosis and radionuclide scans for delineation of the extent of disease. Treatment with nitrogen-containing bisphosphonates is recommended in active disease or when risk of complications is possible. Complications of the disease include arthritis, gait changes, hearing loss, nerve compression syndromes, and osteosarcoma. Total serum alkaline phosphatase is the suggested marker for assessing treatment response when high bone turnover occurs, and it should be measured at three to six months to evaluate initial response. Early diagnosis of Paget disease of bone remains key to its management because patients generally have a good prognosis if treatment is initiated before major complications arise. The primary care physician may need to consult with a specialist for confirmation of diagnosis and initiation of treatment.
佩吉特病是一种良性疾病,其特征是一处或多处骨骼部位的骨转换增加。它通常发生于老年人,男性的风险高于女性。任何骨骼都可能受到影响,但该疾病对骨盆、脊柱、颅骨和长骨有较高的偏好。疼痛是最常见的症状,疾病的表现可能取决于受影响的骨骼、受累的程度以及并发症的存在。佩吉特病可能无症状,在常规血液检查中发现血清碱性磷酸酶水平升高或因其他原因进行影像学检查时偶然发现异常而引起怀疑。循证指南建议使用普通 X 线摄影和血清碱性磷酸酶检测进行初始诊断,并使用放射性核素扫描来描绘疾病的范围。在活动期疾病或存在并发症风险时,建议使用含氮双膦酸盐进行治疗。该疾病的并发症包括关节炎、步态改变、听力损失、神经压迫综合征和骨肉瘤。当发生高骨转换时,总血清碱性磷酸酶是评估治疗反应的建议标志物,应在 3 至 6 个月测量一次以评估初始反应。早期诊断佩吉特病仍然是其管理的关键,因为如果在出现主要并发症之前开始治疗,患者通常预后良好。初级保健医生可能需要咨询专家以确认诊断并开始治疗。