Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis

Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis
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DOI:
10.1016/j.jocn.2008.01.011
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发表时间:
2008-12-01
影响因子:
2
通讯作者:
Mastaglia, Frank L.
Mastaglia, Frank L.
中科院分区:
医学4区
文献类型:
--
作者:
Needham, Merrilee;Corbett, Alastair;Mastaglia, Frank L.

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散发性包涵体肌炎(sIBM)的患病率在不同人群和种族群体中存在差异。 2000 年西澳大利亚州的一项调查发现,每百万人口中患病率为 9.3 例。我们现在进行了一项后续调查,以确定患病率是否有任何变化。目前的患病率为每百万人口 14.9 例,其中 50 岁以上人群的患病率为每百万人口 51.3 例。这是报告的最高 sIBM 患病率,与该人群中 HLA-DR3 和 8.1 主要组织相容性复合体祖先单倍型的高频率相关。对来自三个澳大利亚中心的 57 例 sIBM 病例的综合队列的审查显示,初始误诊率很高,平均诊断时间为 5.2 年,这表明即使是最新的患病率数字也可能被低估,并强调需要提高医学界对该病的认识水平。 (C) 2008 Elsevier Ltd. 保留所有权利。
The prevalence of sporadic inclusion body myositis (sIBM) is variable in different populations and ethnic groups. A previous survey in Western Australia in 2000 found a prevalence of 9.3 per million population. We have now performed a follow-up survey to determine whether there has since been any change in prevalence. The current prevalence was found to be 14.9 per million population, with a prevalence of 51.3 per million population in people over 50 years of age. This is the highest reported prevalence of sIBM and correlates with a high frequency of HLA-DR3 and the 8.1 major histocompatibility complex ancestral haplotype in this population. Review of a combined cohort of 57 sIBM cases from three Australian centres revealed a high rate of initial misdiagnosis and a mean time to diagnosis of 5.2 years, which suggests that even the latest prevalence figure may be an underestimate, and emphasising the need to increase the level of awareness of the condition among the medical community. (C) 2008 Elsevier Ltd. All rights reserved.