Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study
Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study
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DOI：
10.1093/rheumatology/kev240
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发表时间：
2015-12-01
期刊：
影响因子：
5.5
通讯作者：
D'Cruz, David P.
中科院分区：
文献类型：
--
作者：
Hazra, Nisha;Dregan, Alex;D'Cruz, David P.
Objective.复发性多软骨炎是一种罕见的疾病，其特点是软骨炎症。我们的目的是估计复发性多囊卵巢炎的发病率，患病率和死亡率，并描述复发性多囊卵巢炎的临床特点，在一个大的人口。方法.所有被诊断为复发性多囊炎的参与者均从临床实践研究数据链中取样。估计了1990 - 2012年的患病率和发病率。相对死亡率是在一个时间到事件的框架内使用参考英国生命表估计的。一项问卷验证研究评估了诊断准确性。结果有117名参与者记录复发性多囊炎。61例病例中有50例（82%）经医生验证，未证实的病例被排除在外。该分析包括106名被诊断为复发性多囊炎的参与者（42名男性，64名女性）。男性诊断时的平均年龄（范围）为55岁（范围17 - 81），女性为51岁（范围11 - 79）。从首次出现症状到确诊的中位间隔时间为1.9年。1990年至2012年期间复发性多囊卵巢炎的发病率为每年每百万人口0.71例（95% CI 0.55，0.91）。有19人死于各种原因。观察到16例符合生存分析条件的死亡，相同年龄、性别和时期的英国人群预期有7.4例死亡。标化死亡比为2.16（95% CI 1.24，3.51），P< 0.01。呼吸道疾病、心脏病和癌症是最常见的死亡原因。结论复发性多囊炎的发生率可能低于以前的估计，诊断错误分类和延误是常见的。复发性多囊卵巢炎的死亡率是一般人群的两倍多。
Objective. Relapsing polychondritis is a rare disease characterized by cartilage inflammation. Our aim was to estimate the incidence, prevalence and mortality of relapsing polychondritis and describe the clinical features of relapsing polychondritis in a large population. Methods. All participants diagnosed with relapsing polychondritis were sampled from the Clinical Practice Research Datalink. Prevalence and incidence rates for 1990 - 2012 were estimated. Relative mortality rates were estimated in a time- to- event framework using reference UK life tables. A questionnaire validation study assessed diagnostic accuracy. Results. There were 117 participants with relapsing polychondritis ever recorded. Fifty ( 82%) of 61 cases were validated by a physician and unconfirmed cases were excluded. The analysis included 106 participants ( 42 men, 64 women) diagnosed with relapsing polychondritis. The mean age ( range) at diagnosis in men was 55 ( range 17 - 81) years and in women 51 ( range 11 -79) years. The median interval from first symptom to diagnosis was 1.9 years. The incidence of relapsing polychondritis between 1990 and 2012 was 0.71 ( 95% CI 0.55, 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardized mortality ratio was 2.16 ( 95% CI 1.24, 3.51), P< 0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death. Conclusion. The incidence of relapsing polychondritis may be lower than previously estimated, and diagnostic misclassification and delay are common. Mortality in relapsing polychondritis is more than twice that of the general population.