■心脏淀粉样变性(CA)与不良预后相关。筛查研究表明CA被忽视,尤其是在老年人中。治疗的最新进展引起了人们对这种疾病的关注,但是CA流行病学的时间变化数据很少。
■这项工作的目的是描述丹麦的所有CA患者,检查1998年至2017年患者特征的变化。
■从1998年至2017年诊断为任何形式的淀粉样变性的所有患者,以及他们的合并症和药物治疗,在丹麦全国注册管理机构中确定。CA被定义为任何淀粉样变性的诊断代码和心力衰竭的诊断代码。心肌病,或心房纤颤或起搏器植入的程序代码,不管顺序。索引日期被定义为满足这些标准的日期。按索引日期将患者分为5年。为了比较,我们还纳入了普通人群中的对照受试者(比例为1:4).
■619例患者符合CA标准。比较1998-2002年与2013-2017年,基线年龄中位数从67.4岁(四分位距[IQR]:53.9-75.2岁)增加到72.3岁(IQR:66.0-79.3岁)。男性患者的频率从62.1%增加到66.2%。在65岁以上的丹麦人口中,CA的发病率从每100,000人年0.88上升到3.56,2年死亡率从82.6%(IQR:69.9%-90.5%)降至50.2%(IQR:43.1%-56.9%)。与对照组相比,CA患者的死亡率明显较高(log-rank检验:P<0.0001).
■CA,根据这项研究的定义,越来越多地在全国范围内被诊断出来。男性患者的频率和中位年龄的增加表明,野生型甲状腺素运载蛋白淀粉样变性正在推动这种增加。对更早的认识,较少晚期病例可能解释死亡率下降的原因.
UNASSIGNED: Cardiac amyloidosis (CA) has been associated with poor outcomes. Screening studies suggest that CA is overlooked-especially in the elderly. Recent advances in treatment have brought attention to the disease, but data on temporal changes in CA epidemiology are sparse.
UNASSIGNED: The aim of this work was to describe all patients with CA in Denmark, examining changes in patient characteristics from 1998 to 2017.
UNASSIGNED: All patients with any form of amyloidosis diagnosed from 1998 to 2017, as well as their comorbidities and pharmacotherapy, were identified in Danish nationwide registries. CA was defined as any diagnosis code for amyloidosis combined with a diagnosis code for heart failure, cardiomyopathy, or atrial fibrillation or a procedural code for pacemaker implantation, regardless of the order. The index date was defined as the date of meeting those criteria. Patients were divided into 5-year periods by index date. For comparison, we also included control subjects (1:4 ratio) from the general population.
UNASSIGNED: CA criteria were met by 619 patients. Comparing 1998-2002 vs 2013-2017, the median age at baseline increased from 67.4 years (interquartile range [IQR]: 53.9-75.2 years) to 72.3 years (IQR: 66.0-79.3 years). The frequency of male patients increased from 62.1% to 66.2%. The incidence of CA rose from 0.88 to 3.56 per 100,000 person-years in the Danish population aged ≥65 years, and the 2-year mortality decreased from 82.6% (IQR: 69.9%-90.5%) to 50.2% (IQR: 43.1%-56.9%). Compared with control subjects, the mortality among CA patients was significantly higher (log-rank test: P < 0.0001).
UNASSIGNED: CA, as defined in this study, was increasingly diagnosed on a national scale. The increasing frequency of male patients and median age suggest that wild-type transthyretin amyloidosis is driving this increase. Greater recognition of earlier, less advanced cases might explain decreasing mortality.