Abstract：
BACKGROUND: Mastocytosis is a disease characterized by accumulation of aberrant mast cells and mediator-related symptoms and is divided into systemic mastocytosis (SM) and cutaneous mastocytosis (CM). The epidemiology of mastocytosis remains incompletely understood.
OBJECTIVE: To estimate the incidence, prevalence, overall survival (OS) and burden of comorbidities in adult mastocytosis patients identified in Swedish population-based registries.
METHODS: Individuals (≥ 20 years of age) with a mastocytosis diagnosis in the National Patient Register (NPR) and/or the Swedish Cancer Register (SCR) between 2001 and 2018, were identified. In a matched cohort design, for each case five randomly selected mastocytosis-free comparators matched on age, sex, and county of residence were chosen from the Population Register. The Kaplan-Meier method was used to compare OS between individuals with mastocytosis and comparators. Information on concomitant disease at baseline was assessed by use of the Charlson Comorbidity Index (CCI).
RESULTS: We identified 2,040 adults with a mastocytosis diagnosis yielding an annual incidence of 1.56 per 100,000 (95% CI 1.29-1.87) and a prevalence of 23.9 per 100,000 (95% CI 22.8-25.0). The comorbidity burden was higher, and the OS lower, in patients with mastocytosis compared to comparators.
CONCLUSIONS: We found a higher incidence and prevalence of mastocytosis compared to assessments in other settings and confirmed that the prognosis generally is favorable. Of special note was evidence of a higher comorbidity burden in mastocytosis patients compared to the background population.
CONCLUSIONS: Underreporting and inconsistencies in the use of diagnostic codes.
OBJECTIVE: To estimate the incidence, prevalence, overall survival (OS) and burden of comorbidities in adult mastocytosis patients identified in Swedish population-based registries.
METHODS: Individuals (≥ 20 years of age) with a mastocytosis diagnosis in the National Patient Register (NPR) and/or the Swedish Cancer Register (SCR) between 2001 and 2018, were identified. In a matched cohort design, for each case five randomly selected mastocytosis-free comparators matched on age, sex, and county of residence were chosen from the Population Register. The Kaplan-Meier method was used to compare OS between individuals with mastocytosis and comparators. Information on concomitant disease at baseline was assessed by use of the Charlson Comorbidity Index (CCI).
RESULTS: We identified 2,040 adults with a mastocytosis diagnosis yielding an annual incidence of 1.56 per 100,000 (95% CI 1.29-1.87) and a prevalence of 23.9 per 100,000 (95% CI 22.8-25.0). The comorbidity burden was higher, and the OS lower, in patients with mastocytosis compared to comparators.
CONCLUSIONS: We found a higher incidence and prevalence of mastocytosis compared to assessments in other settings and confirmed that the prognosis generally is favorable. Of special note was evidence of a higher comorbidity burden in mastocytosis patients compared to the background population.
CONCLUSIONS: Underreporting and inconsistencies in the use of diagnostic codes.
摘要：
背景：肥大细胞增多症是一种以肥大细胞异常积累和介质相关症状为特征的疾病，分为系统性肥大细胞增多症（SM）和皮肤肥大细胞增多症（CM）。肥大细胞增多症的流行病学仍未完全了解。
目的：为了估计发病率，患病率,在瑞典以人群为基础的登记中发现的成年肥大细胞增多症患者的总生存期(OS)和合并症负担.
方法：确定了2001年至2018年在国家患者登记册（NPR）和/或瑞典癌症登记册（SCR）中诊断为肥大细胞增多症的个体（≥20岁）。在匹配的队列设计中，对于每种情况，五个随机选择的无肥大细胞病比较器在年龄上匹配，性别,和居住县是从人口登记册中选择的。Kaplan-Meier方法用于比较肥大细胞增多症患者和比较者之间的OS。通过使用Charlson合并症指数（CCI）评估基线时伴随疾病的信息。
结果：我们确定了2,040名诊断为肥大细胞增多症的成年人，年发病率为1.56/100,000（95％CI1.29-1.87），患病率为23.9/100,000（95％CI22.8-25.0）。共病负担较高，操作系统更低，与比较者相比，肥大细胞增多症患者。
结论：我们发现与其他环境的评估相比，肥大细胞增多症的发生率和患病率更高，并证实预后总体良好。特别值得注意的是，与背景人群相比，肥大细胞增多症患者的合并症负担更高。
结论：诊断代码的使用存在漏报和不一致的问题。
目的：为了估计发病率，患病率,在瑞典以人群为基础的登记中发现的成年肥大细胞增多症患者的总生存期(OS)和合并症负担.
方法：确定了2001年至2018年在国家患者登记册（NPR）和/或瑞典癌症登记册（SCR）中诊断为肥大细胞增多症的个体（≥20岁）。在匹配的队列设计中，对于每种情况，五个随机选择的无肥大细胞病比较器在年龄上匹配，性别,和居住县是从人口登记册中选择的。Kaplan-Meier方法用于比较肥大细胞增多症患者和比较者之间的OS。通过使用Charlson合并症指数（CCI）评估基线时伴随疾病的信息。
结果：我们确定了2,040名诊断为肥大细胞增多症的成年人，年发病率为1.56/100,000（95％CI1.29-1.87），患病率为23.9/100,000（95％CI22.8-25.0）。共病负担较高，操作系统更低，与比较者相比，肥大细胞增多症患者。
结论：我们发现与其他环境的评估相比，肥大细胞增多症的发生率和患病率更高，并证实预后总体良好。特别值得注意的是，与背景人群相比，肥大细胞增多症患者的合并症负担更高。
结论：诊断代码的使用存在漏报和不一致的问题。
