A nationwide study of the epidemiology of relapsing polychondritis
A nationwide study of the epidemiology of relapsing polychondritis
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DOI：
10.2147/clep.s91439
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发表时间：
2016-01-01
影响因子：
3.9
通讯作者：
Pollner, Peter
中科院分区：
文献类型：
--
作者：
Horvath, Anna;Pall, Nora;Pollner, Peter
目的：复发性多软骨炎（RP）是一种罕见的自身免疫性炎症性疾病，主要侵袭软骨结构或对富含蛋白多糖的结构（眼、心、血管、内耳）造成严重损害。这项研究通过收集中欧全国1.24亿人的所有病例，显示了有关这种高度可变疾病的流行病学、进展和关联的结果。方法：我们使用匈牙利卫生保健数据库来识别所有可能的RP感染患者。我们跟踪了2002年1月1日至2013年12月31日期间匈牙利住院或门诊记录中至少一次具有国际疾病分类第10版代码M94.1的患者。我们根据患者在2010年1月1日至2013年12月31日期间的药物消费模式将这些患者分为疾病严重程度组。我们还分析了RP发病率的区域分布。共病的总体地图以网络布局呈现。结果：我们在累计1150万登记居民中确定了256例RP患者。我们将这些患者分为“极轻度”（n=144）、“轻度”（n=22）、“中度”（n=41）和“重度”（n=4）四个严重级别。另外两组为没有可用药物数据的患者定义为“仅怀疑”（n=23）和“确认但未知治疗”（n=22）。患者的年龄和性别分布与世界统计数据相似。事实上，总体生存率很好（5年95%置信区间为83.6%-92.9%，10年为75.0%-88.3%，这与匈牙利一般人群的总体生存率相对应），并且与匈牙利其他自身免疫性疾病的相关性很高（56%）。几乎任何疾病都可能发生于RP；然而，染色体异常的症状只是偶然的。颈椎病可能是RP激活的标志，而干燥综合征是最常见的自身免疫性关联。发病率的区域分布表明，饮用水和阳光照射可能是引发砷中毒的因素。结论：匈牙利RP的良好生存率可能与该病的早期诊断有关。
Objective: Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort.Methods: We used the Hungarian Health Care Database to identify all persons with possible RP infection. We followed patients who had International Classification of Diseases 10th edition code M94.1 at least once in their inpatient or outpatient records between January 1, 2002 and December 31, 2013 in Hungary. We classified these patients into disease severity groups by their drug consumption patterns between January 1, 2010 and December 31, 2013. We analyzed the regional distribution of RP incidences as well. Overall maps of comorbidity are presented with network layouts.Results: We identified 256 patients with RP among cumulatively 11.5 million registered inhabitants. We classified these patients into four severity classes as "extremely mild" (n=144), "mild" (n=22), "moderate" (n=41), and "severe" (n=4). Two additional groups were defined for patients without available drug data as "suspected only" (n=23) and "confirmed but unknown treatment" (n=22). The age and sex distributions of patients were similar to worldwide statistics. Indeed, the overall survival was good (95% confidence interval for 5 years was 83.6%-92.9% and for 10 years was 75.0%-88.3% which corresponds to the overall survival of the general population in Hungary), and the associations with other autoimmune disorders were high (56%) in Hungary. Almost any disease can occur with RP; however, the symptoms of chromosomal abnormalities are only incidental. Spondylosis can be a sign of the activation of RP, while Sjogren syndrome is the most frequent autoimmune association. Regional distribution of incidences suggests arsenic drinking water and sunlight exposure as possible triggering factors.Conclusion: The good survival rate of RP in Hungary is probably associated with the early diagnosis of the disease.