Dobloug G C, Antal E A, Sveberg L, Garen T, Bitter H, Stjärne J, Grøvle L, Gran J T, Molberg Ø
Department of Rheumatology, Oslo University Hospital (OUH), Oslo, Norway.
Eur J Neurol. 2015 Apr;22(4):672-e41. doi: 10.1111/ene.12627. Epub 2014 Dec 21.
Knowledge about the occurrence of sporadic inclusion body myositis (sIBM) in the general population is limited. Here, our aim was to identify and characterize every sIBM patient living in southeast Norway (population 2.64 million) from 2003 to 2012.
Two sIBM case finding strategies were applied. First, all hospital databases in southeast Norway were screened to identify cases with sIBM-compatible International Classification of Diseases 10 (ICD-10) codes. These cases were then manually chart reviewed. Secondly, all muscle histology reports encoded with inflammation were independently reviewed. Finally, cases were classified according to the 1997 and the 2011 European Neuro-Muscular Centre (ENMC) Research Diagnostic Criteria for sIBM.
The combined case finding strategy identified 3160 patients with sIBM compatible ICD-10 codes, and a largely overlapping cohort of 500 patients having muscle biopsies encoded with inflammation. Detailed retrospective review of chart and histology data showed that 95 patients met the 2011 ENMC sIBM criteria and 92 met the 1997 criteria. Estimated point prevalence of sIBM was 33/1 000 000, equal with both criteria sets. Mean age at diagnosis was 66.9 years and mean diagnostic delay was 5.6 years. Chart review revealed higher frequencies of dysphagia (94% vs. 65%) and anti-Sjøgren syndrome A antibodies (39% vs. 12%) in female sIBM patients (n = 40) than in males. Coexisting rheumatic diseases were present in 25% of sIBM cases, with Sjøgren's syndrome in 10%.
An estimated point prevalence of sIBM seven times higher than previously observed in Europe is reported. Our data show considerable diagnostic delay, a major challenge with new sIBM treatments in the pipeline.
关于散发性包涵体肌炎（sIBM）在普通人群中的发病情况，相关知识有限。在此，我们的目的是识别并描述2003年至2012年居住在挪威东南部（人口264万）的每一位sIBM患者。
应用了两种sIBM病例发现策略。首先，筛查挪威东南部所有医院数据库，以识别具有与sIBM相符的国际疾病分类第10版（ICD - 10）编码的病例。然后对这些病例进行人工病历审查。其次，对所有编码为炎症的肌肉组织学报告进行独立审查。最后，根据1997年和2011年欧洲神经肌肉中心（ENMC）的sIBM研究诊断标准对病例进行分类。
联合病例发现策略识别出3160例具有与sIBM相符的ICD - 10编码的患者，以及一个在很大程度上重叠的队列，其中500例患者的肌肉活检编码为炎症。对病历和组织学数据的详细回顾性审查显示，95例患者符合2011年ENMC的sIBM标准，92例符合1997年标准。sIBM的估计时点患病率为33/1000000，两种标准集的结果相同。诊断时的平均年龄为66.9岁，平均诊断延迟为5.6年。病历审查显示，女性sIBM患者（n = 40）吞咽困难（94%对65%）和抗干燥综合征A抗体（39%对12%）的出现频率高于男性。25%的sIBM病例存在合并的风湿性疾病，其中10%为干燥综合征。
报告的sIBM估计时点患病率比欧洲此前观察到的高出7倍。我们的数据显示存在相当大的诊断延迟，这是即将推出的新型sIBM治疗方法面临的一项重大挑战。
