Epidemiology and outcome of adult-onset Still's disease in Northern Norway
Epidemiology and outcome of adult-onset Still's disease in Northern Norway
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DOI：
10.1080/03009740510026616
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发表时间：
2006-02-01
影响因子：
2.1
通讯作者：
Nossent, HC
中科院分区：
文献类型：
--
作者：
Nossent, HC
背景：成人发病的斯蒂尔斯病（AOSD）被认为是一种罕见的疾病，但关于AOSD的发病率和患病率的数据很少。本研究分析了挪威北部稳定同质人群中AOSD的流行病学、疾病表现和预后。方法：对1990 - 2000年在该地区唯一一家设有风湿病科的医院登记的所有AOSD患者进行回顾性队列研究。临床诊断和排除患者以山口AOSD标准为指导。基线时的人口学和临床数据从患者记录中提取，并辅以对照就诊时收集的数据。数据分析采用非参数方法。结果：确诊AOSD 13例；估计AOSD的年发病率为0.4/10万成人（95% CI 0.11 - 0.97），而AOSD的点患病率从1990年的3.4/10万（95% CI 0.8 - 9.4）增加到2000年的6.9/ 10万（95% CI 2.7 - 14.2）。平均诊断延迟为5.2个月（0.5 - 18个月）。血清铁蛋白bbb50为正常上水平的5倍，诊断敏感性为63%。在69个月的随访中，1例患者死亡，6/13例患者获得持续缓解，而6例患者出现慢性进展（n=3）或复发/缓解病程（n=3）。这六项专利中有四项必须进入社会保障计划。结论：挪威北部AOSD的年发病率至少为0.4/10万。该地区的AOSD比法国或日本更普遍，影响更多的男性，患病率接近青少年斯蒂尔氏病。所有患者中有一半为单环病程，而慢性疾病患者中发生死亡和残疾。
Background: Adult-onset Still's disease (AOSD) is considered a rare disease, but few data exist on the incidence and prevalence of AOSD. This study has analysed the epidemiology, disease presentation, and outcome of AOSD in a stable homogeneous population in Northern Norway.Methods: A retrospective cohort study of all AOSD patients registered in 1990 - 2000 at the only hospital in the region with a Rheumatology Service. Clinical diagnosis and exclusion of patients were directed by the Yamaguchi criteria for AOSD. Demographic and clinical data at baseline were extracted from patient records and supplemented with data gathered at control visits. Data were analysed with nonparametric methods.Results: AOSD was ascertained in 13 patients; the estimated annual AOSD incidence was 0.4/100 000 adults (95% CI 0.11 - 0.97), while point prevalence of AOSD increased from 3.4/100 000 ( 95% CI 0.8 - 9.4) in 1990 to 6.9/ 100 000 in 2000 ( 95% CI 2.7 - 14.2). Mean diagnostic delay was 5.2 months ( range 0.5 - 18). Serum ferritin >5 times the normal upper level had 63% diagnostic sensitivity. During 69 months' follow-up, one patient died, 6/13 patients achieved sustained remission, while six patients developed a chronic progressive (n=3) or a relapsing/remitting disease course (n=3). Four of these six patents had to enter social security programmes.Conclusion: The annual incidence of AOSD in Northern Norway is at least 0.4/100 000 adults. AOSD in this region is more prevalent than in France or Japan, affects more males, and approximates to the prevalence of juvenile Still's disease. Half of all patients have a monocyclic disease course, while mortality and invalidity occur in patients with chronic disease.