Merlotti Daniela, Gennari Luigi, Martini Giuseppe, Nuti Ranuccio
Department of Internal Medicine, Endocrine-Metabolic Sciences and Biochemistry, University of Siena, Siena, Italy.
Open Access Rheumatol. 2009 Jul 17;1:107-120. doi: 10.2147/oarrr.s4504. eCollection 2009.
Paget's disease of bone (PDB) is a chronic bone remodelling disorder characterized by increased osteoclast-mediated bone resorption, with subsequent compensatory increases in new bone formation, resulting in a disorganized mosaic of woven and lamellar bone at affected skeletal sites. This disease is most often asymptomatic but can be associated with bone pain or deformity, fractures, secondary arthritis, neurological complications, deafness, contributing to substantial morbidity and reduced quality of life. Neoplastic degeneration of pagetic bone is a relatively rare event, occurring with an incidence of less than 1%, but has a grave prognosis. Specific therapy for PDB is aimed at decreasing the abnormal bone turnover and bisphosphonates are currently considered the treatment of choice. These treatments are associated with a reduction in plasma alkaline phosphatase (ALP) activity and an improvement in radiological and scintigraphic appearance and with a reduction in bone pain and bone deformity, Recently, the availability of newer, more potent nitrogen-containing bisphosphonates has improved treatment outcomes, allowing a more effective and convenient management of this debilitating disorder.
骨佩吉特病（PDB）是一种慢性骨重塑紊乱疾病，其特征为破骨细胞介导的骨吸收增加，随后新骨形成出现代偿性增加，导致受累骨骼部位出现编织骨和板层骨的杂乱镶嵌。这种疾病通常无症状，但可能与骨痛或畸形、骨折、继发性关节炎、神经并发症、耳聋有关，会导致严重的发病率并降低生活质量。佩吉特骨的肿瘤性退变是一种相对罕见的情况，发生率低于1%，但预后严重。PDB的特异性治疗旨在减少异常的骨转换，双膦酸盐目前被认为是首选治疗方法。这些治疗与血浆碱性磷酸酶（ALP）活性降低、放射学和骨闪烁显像表现改善以及骨痛和骨畸形减轻有关。最近，更新的、更有效的含氮双膦酸盐的出现改善了治疗效果，使这种使人衰弱的疾病得到更有效和方便的管理。
