Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark.
Shire US Inc., a Takeda company, Lexington, MA, USA.
Haemophilia. 2021 Mar;27(2):277-282. doi: 10.1111/hae.14257. Epub 2021 Feb 6.
Information about temporal development of von Willebrand disease (VWD) incidence at a population level is scarce. To our knowledge, no study has described the incidence of VWD at a population level.
To estimate overall and annual incidence rates of hospital diagnosed VWD in Denmark between 1995 and 2016 as well as the frequency of hospital treated bleeding episodes before and after VWD diagnosis.
A registry-based cohort study that included all Danish patients with a first diagnosis of VWD in Denmark, identified in the Danish National Patient Registry through 1995-2016.
We identified 1,035 patients with a diagnosis of VWD. The overall incidence rate of VWD in 1995-2016 was 8.6 (95% CI: 8.1-9.2). The annual age-standardized incidence rate per 100 000 person-years varied between 4.1 (95% CI: 2.4-5.9) in 1998 and 16.7 (95% CI: 13.1-20.3) in 2005. A prominent peak in rates appeared from 2002 to 2008. One and five years before VWD diagnosis, 6% and 11.5% of the patients had at least one hospital treated bleeding episode. One and five years after diagnosis, the corresponding percentages were 7.9% and 13.4%.
These results are the first population-based estimates of VWD incidence. The incidence may be underestimated because asymptomatic individuals may not be diagnosed. The observed peak in incidence from 2002-2008 may be explained by increased medical attention, leading to more patients being diagnosed, rather than an actual increase in VWD incidence. However, overall, we observed no systematic changes in VWD incidence over the study period.
关于人群中 von Willebrand 病（VWD）发病率的时间发展的信息很少。据我们所知，尚无研究描述人群中 VWD 的发病率。
评估 1995 年至 2016 年丹麦人群中确诊 VWD 的总体和年度发病率，以及 VWD 诊断前后医院治疗的出血发作频率。
这是一项基于登记的队列研究，纳入了在丹麦国家患者登记处通过 1995-2016 年首次诊断为 VWD 的所有丹麦患者。
我们确定了 1035 例 VWD 患者。1995-2016 年 VWD 的总体发病率为 8.6（95%CI：8.1-9.2）。每 100000 人年的年龄标准化发病率在 1998 年为 4.1（95%CI：2.4-5.9），在 2005 年为 16.7（95%CI：13.1-20.3）。发病率出现了一个明显的高峰，出现在 2002 年至 2008 年期间。VWD 诊断前 1 年和 5 年，有 6%和 11.5%的患者至少有一次医院治疗的出血发作。诊断后 1 年和 5 年，相应的百分比分别为 7.9%和 13.4%。
这些结果是人群中 VWD 发病率的首次基于人群的估计。由于无症状个体可能未被诊断，因此发病率可能被低估。2002-2008 年发病率的观察到的高峰可能是由于医疗关注度的增加导致更多患者被诊断出来，而不是 VWD 发病率的实际增加。然而，总体而言，我们在研究期间未观察到 VWD 发病率的系统性变化。
