Smith Stacy E, Murphey Mark D, Motamedi Kambiz, Mulligan Michael E, Resnik Charles S, Gannon Francis H
Department of Radiology, University of Maryland School of Medicine, Baltimore, USA.
Radiographics. 2002 Sep-Oct;22(5):1191-216. doi: 10.1148/radiographics.22.5.g02se281191.
Paget disease of bone is a common disorder affecting approximately 3%-4% of the population over 40 years of age. The pathologic abnormality in Paget disease is excessive and abnormal remodeling of bone. Three pathologic phases have been described: the lytic phase (incipient-active), in which osteoclasts predominate; the mixed phase (active), in which osteoblasts cause repair superimposed on the resorption; and the blastic phase (late-inactive) in which osteoblasts predominate. Radiographic appearance of Paget disease reflects these pathologic changes and is often characteristic. Initially, there is osteolysis, particularly affecting the skull (osteoporosis circumscripta) and subchondral long bones, with subsequent development of trabecular and cortical thickening and enlargement of bone in the mixed phase of the disease. Finally, areas of sclerosis may develop in the blastic phase. Frequent sites of involvement include the skull (25%-65% of cases), spine (30%-75%), pelvis (30%-75%), and proximal long bones (25%-30%). Bone scintigraphy typically demonstrates marked increased uptake of radionuclide in all phases of Paget disease. Computed tomography and magnetic resonance imaging often show changes similar to those seen radiographically in noncomplicated Paget disease with maintenance of yellow marrow. Complications of Paget disease include the effects of osseous weakening (deformity and fracture), arthritis, neurologic symptoms, and neoplastic involvement. Sarcomatous transformation is the most feared complication, occurring in approximately 1% of cases, and is seen on images as focal bone destruction extending through the cortex with an associated soft-tissue mass. Recognition of the radiologic spectrum of the appearances of Paget disease usually allows prospective diagnosis and differentiation of its associated complications, which helps guide therapy and improve patient management.
骨佩吉特病是一种常见疾病，影响约3%-4%的40岁以上人群。佩吉特病的病理异常是骨的过度和异常重塑。已描述了三个病理阶段：溶骨期（初期活跃期），以破骨细胞为主；混合期（活跃期），成骨细胞在吸收的基础上进行修复；以及骨硬化期（晚期静止期），以成骨细胞为主。佩吉特病的影像学表现反映了这些病理变化，且通常具有特征性。最初，存在骨质溶解，尤其影响颅骨（局限性骨质疏松）和软骨下长骨，随后在疾病的混合期出现小梁和皮质增厚以及骨增大。最后，在骨硬化期可能出现硬化区域。常见受累部位包括颅骨（25%-65%的病例）、脊柱（30%-75%）、骨盆（30%-75%）和近端长骨（25%-30%）。骨闪烁显像通常显示在佩吉特病的所有阶段放射性核素摄取明显增加。计算机断层扫描和磁共振成像常显示与非复杂性佩吉特病的影像学表现相似的变化，并伴有黄骨髓。佩吉特病的并发症包括骨质减弱的影响（畸形和骨折）、关节炎、神经症状和肿瘤累及。肉瘤样变是最可怕的并发症，约1%的病例会发生，在影像上表现为穿过皮质的局灶性骨质破坏并伴有相关软组织肿块。认识佩吉特病的影像学表现谱通常有助于前瞻性诊断及其相关并发症的鉴别，这有助于指导治疗并改善患者管理。
