文章：

骨保护素是原发性系统性淀粉样变性患者总生存期的重要预后因素，独立于Mayo分期

Osteoprotegerin is a significant prognostic factor for overall survival in patients with primary systemic amyloidosis independent of the Mayo staging

原文发布日期：2015-06-05

DOI: 10.1038/bcj.2015.45

类型: Original Article

开放获取: 是

英文摘要：

Bone metabolism has not been systematically studied in primary (AL) amyloidosis. Thus we prospectively evaluated bone remodeling indices in 102 patients with newly diagnosed AL amyloidosis, 35 healthy controls, 35 newly diagnosed myeloma and 40 monoclonal gammopathy of undetermined significance patients. Bone resorption markers (C-telopeptide of type-1 collagen, N-telopeptide of type-1 collagen) and osteoclast regulators (soluble receptor activator of nuclear factor-κB ligand (sRANKL), osteoprotegerin (OPG)) were increased in AL patients compared with controls (P<0.01), but bone formation was unaffected. Myeloma patients had increased bone resorption and decreased bone formation compared with AL patients, while sRANKL/OPG ratio was markedly decreased in AL, due to elevated OPG in AL (P<0.001). OPG correlated with N-terminal pro-brain natriuretic peptide (P<0.001) and was higher in patients with cardiac involvement (P=0.028) and advanced Mayo stage (P=0.001). OPG levels above the upper value of healthy controls was associated with shorter survival (34 versus 91 months; P=0.026), while AL patients with OPG levels in the top quartile had very short survival (12 versus 58 months; P=0.024). In Mayo stage 1 disease, OPG identified patients with poor survival (12 versus >60 months; P=0.012). We conclude that increased OPG in AL is not only a compensation to osteoclast activation but may also reflect early cardiac damage and may identify patients at increased risk of death within those with earlier Mayo stage.

摘要翻译： 

原发性轻链型淀粉样变性中的骨代谢尚未得到系统性研究。为此，我们前瞻性评估了102例新诊断AL淀粉样变性患者、35例健康对照者、35例新诊断多发性骨髓瘤患者及40例意义未明单克隆丙种球蛋白病患者的骨重塑指标。与对照组相比，AL患者的骨吸收标志物（Ⅰ型胶原C端肽、Ⅰ型胶原N端肽）和破骨细胞调节因子（可溶性核因子κB受体活化因子配体、骨保护素）均升高（P<0.01），但骨形成未受影响。与AL患者相比，骨髓瘤患者骨吸收增加且骨形成减少，而由于AL患者OPG升高（P<0.001），其sRANKL/OPG比值显著降低。OPG与N末端前脑钠肽呈正相关（P<0.001），在心脏受累患者（P=0.028）及梅奥分期晚期患者（P=0.001）中水平更高。OPG水平高于健康对照组上限的患者生存期更短（34个月 vs 91个月；P=0.026），而OPG水平处于最高四分位数的AL患者生存期极短（12个月 vs 58个月；P=0.024）。在梅奥分期1期患者中，OPG可识别生存期较差的患者（12个月 vs >60个月；P=0.012）。我们得出结论：AL患者OPG升高不仅是对破骨细胞活化的代偿反应，还可能反映早期心脏损伤，并可在梅奥分期较早的患者中识别死亡风险增高的人群。

原文链接：

Osteoprotegerin is a significant prognostic factor for overall survival in patients with primary systemic amyloidosis independent of the Mayo staging