1 Department of Health Research and Policy, Stanford School of Medicine, Stanford, CA, USA.
2 Clinical Epidemiology Unit, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden.
Public Health Rep. 2018 Jul/Aug;133(4):481-488. doi: 10.1177/0033354918777253. Epub 2018 Jun 21.
Mortality due to rare diseases, which are substantial sources of premature mortality, is underreported in mortality studies. The objective of this study was to determine the completeness of reporting systemic lupus erythematosus (SLE) as a cause of death.
In 2017, we linked data on a Swedish population-based cohort (the Swedish Lupus Linkage, 2001-2013) comprising people with SLE (n = 8560) and their matched general population comparators (n = 37 717) to data from the Cause of Death Register. We reviewed death records of deceased people from the cohort (n = 5110) and extracted data on patient demographic characteristics and causes of death. We estimated odds ratios (ORs) and 95% confidence intervals (CIs) for not reporting SLE as a cause of death by using multivariable-adjusted logistic regression models.
Of 1802 deaths among SLE patients in the study, 1071 (59%) did not have SLE reported on their death records. Most SLE decedents were aged 75-84 at death (n = 584, 32%), female (n = 1462, 81%), and born in Nordic countries (n = 1730, 96%). Decedents aged ≥85 at death were more likely to have SLE not reported on their death records than were decedents aged <50 (OR = 2.34; 95% CI, 1.48-3.68). Having renal failure listed as a cause of death decreased the likelihood of SLE not being reported on the death record (OR = 0.54; 95% CI, 0.40-0.73), whereas having cancer listed as a cause of death increased this likelihood (OR = 2.39; 95% CI, 1.85-3.07).
SLE was greatly underreported as a cause of mortality on death records of SLE patients, particularly in older decedents and those with cancer, thereby underestimating the true burden of this disease. Public health resources need to focus on improving the recording of rare diseases in order to enhance the epidemiological utility of mortality data.
由于罕见疾病是导致过早死亡的主要原因之一，因此在死亡率研究中，这些疾病的死亡率报告并不完整。本研究的目的是确定系统性红斑狼疮（SLE）作为死亡原因的报告是否完整。
2017 年，我们将一个基于瑞典人群的队列（瑞典狼疮关联研究，2001-2013 年）的数据与死因登记处的数据进行了链接，该队列包括 8560 例 SLE 患者（n=8560）和 37717 名匹配的普通人群对照者（n=37717）。我们查阅了队列中死亡患者（n=5110）的死亡记录，并提取了患者人口统计学特征和死因的数据。我们使用多变量调整后的逻辑回归模型来估计未将 SLE 报告为死亡原因的可能性比（ORs）和 95%置信区间（CIs）。
在研究中的 1802 例 SLE 患者死亡中，有 1071 例（59%）的死亡记录中未报告 SLE。大多数 SLE 死者在死亡时年龄为 75-84 岁（n=584，32%），女性（n=1462，81%），出生于北欧国家（n=1730，96%）。年龄≥85 岁的死者报告 SLE 死亡记录的可能性高于年龄<50 岁的死者（OR=2.34；95%CI，1.48-3.68）。将肾衰竭列为死因会降低 SLE 未被报告为死亡记录的可能性（OR=0.54；95%CI，0.40-0.73），而将癌症列为死因会增加这种可能性（OR=2.39；95%CI，1.85-3.07）。
SLE 在 SLE 患者的死亡记录中作为死亡原因的报告严重不足，尤其是在年龄较大的死者和患有癌症的死者中，从而低估了这种疾病的真实负担。公共卫生资源需要集中精力改善罕见疾病的记录，以提高死亡率数据的流行病学实用性。
