University of Maryland School of Medicine, 800 W. Baltimore Street, Baltimore, MD 21201, USA.
Am J Pathol. 2010 Jul;177(1):280-90. doi: 10.2353/ajpath.2010.090592. Epub 2010 May 14.
Bisphosphonate-associated osteonecrosis of the jaw (BONJ) is a morbid bone disease linked to long-term bisphosphonate use. Despite its broad health impact, mechanistic study is lacking. In this study, we have established a mouse model of BONJ-like disease based on the equivalent clinical regimen in myeloma patients, a group associated with high risk of BONJ. We demonstrate that the murine BONJ-like disease recapitulates major clinical and radiographical manifestations of the human disease, including characteristic features of osseous sclerosis, sequestra, avascular, and radiopaque alveolar bone in the jaw that persists beyond a normal course of wound healing following tooth extraction. We find that long-term administration of bisphosphonates results in an increase in the size and number of osteoclasts and the formation of giant osteoclast-like cells within the alveolar bone. We show that the development of necrotic bone and impaired soft tissue healing in our mouse model is dependent on long-term use of high-dose bisphosphonates, immunosuppressive and chemotherapy drugs, as well as mechanical trauma. Most importantly, we demonstrate that bisphosphonate is the major cause of BONJ-like disease in mice, mediated in part by its ability to suppress osseous angiogenesis and bone remodeling. The availability of this novel mouse model of BONJ-like disease will help elucidate the pathophysiology of BONJ and ultimately develop novel approaches for prevention and treatment of human BONJ.
颌骨骨坏死(BONJ)与长期使用双膦酸盐有关,是一种病态的骨疾病。尽管它对健康有广泛的影响,但缺乏机制研究。在这项研究中,我们根据骨髓瘤患者的等效临床方案建立了类似于 BONJ 的疾病的小鼠模型,骨髓瘤患者是 BONJ 高风险人群。我们证明,类似于 BONJ 的小鼠疾病再现了人类疾病的主要临床和影像学表现,包括颌骨骨硬化、死骨、无血管和不透射线的牙槽骨的特征,这些特征在拔牙后正常愈合过程之外仍然存在。我们发现,长期使用双膦酸盐会导致破骨细胞的大小和数量增加,并在牙槽骨内形成巨大的破骨细胞样细胞。我们表明,我们的小鼠模型中坏死骨的形成和软组织愈合受损依赖于长期使用大剂量双膦酸盐、免疫抑制和化疗药物以及机械创伤。最重要的是,我们证明双膦酸盐是导致类似于 BONJ 的疾病的主要原因,部分原因是其抑制骨血管生成和骨重塑的能力。这种新型类似于 BONJ 的疾病的小鼠模型的可用性将有助于阐明 BONJ 的病理生理学,并最终开发出预防和治疗人类 BONJ 的新方法。