Idiopathic chronic inflammatory demyelinating polyneuropathy:: an epidemiological study in Italy
Idiopathic chronic inflammatory demyelinating polyneuropathy:: an epidemiological study in Italy
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DOI：
10.1136/jnnp.2007.114868
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发表时间：
2007-12-01
影响因子：
11
通讯作者：
Calvo, A.
中科院分区：
文献类型：
--
作者：
Chio, A.;Cocito, D.;Calvo, A.
目的：评估意大利人群慢性炎症性脱髓鞘性多神经病变（CIDP）的临床和流行病学特征。对象和方法：所有1990年后在Piemonte和Valle d'Aosta诊断为脱髓鞘性神经病变的受试者（4 334 225名居民）。根据美国神经病学学会的研究标准诊断CIDP， 294例患者中有165例符合诊断标准。结果：粗患病率为3.58/ 10万人（95% CI 3.02 ~ 4.20）。发病当日，确诊76例（49.0%），可能67例（43.2%），可能12例（7.7%）；轻度残疾105例（67.7%），中度残疾32例（20.6%），重度残疾18例（11.6%）。病程为缓解-复发型40例（25.8%），慢性进行性96例（61.9%），单相19例（12.3%）。1995-2001年95例糖尿病患者，年平均粗发病率为0.36/10万人（95% CI 0.29 ~ 0.44），男女比例为2.3:1.14例糖尿病患者。在多变量分析中，流行日与严重残疾相关的因素有：年龄。60年;诊断时免疫调节治疗失败；更严重的残疾在最低点；慢性病程。结论：意大利CIDP的发病率和患病率高于以往大多数研究。在流行日，超过80%的病例有轻度或中度残疾，这表明要么对免疫调节治疗有良好的反应，要么在大多数病例中CIDP有轻度病程的趋势。
Aim: The clinical and epidemiological characteristics of chronic inflammatory demyelinating polyneuropathy (CIDP) in an Italian population were assessed.Subjects and methods: All subjects with a diagnosis of demyelinating neuropathy after 1990 in Piemonte and Valle d'Aosta (4 334 225 inhabitants) were considered. The diagnosis of CIDP was based on the research criteria of the American Academy of Neurology. 165 of 294 patients met the diagnostic criteria.Results: The crude prevalence rate was 3.58/ 100 000 population (95% CI 3.02 to 4.20). At the prevalence day, 76 (49.0%) cases had definite, 67 (43.2%) probable and 12 (7.7%) possible CIDP; disability was mild in 105 (67.7%) cases, moderate in 32 (20.6%) and severe in 18 (11.6%). The course was remitting-relapsing in 40 cases (25.8%), chronic progressive in 96 (61.9%) and monophasic in 19 (12.3%). Considering the 95 patients whose disorder presented in the period 1995-2001, the mean annual crude incidence rate was 0.36/100 000 population (95% CI 0.29 to 0.44), with a male to female ratio of 2.3: 1.14 cases were affected by diabetes mellitus. In multivariate analysis, factors related to severe disability at the prevalence day were: age. 60 years; failure of immunomodulating therapies at the time of diagnosis; worse disability at nadir; and chronic course.Conclusion: Incidence and prevalence rates of CIDP in Italy were higher than those observed in most previous studies. At the prevalence day, more than 80% of cases had a mild or moderate disability, indicating either a good response to immunomodulating therapy or a tendency of CIDP to have a mild course in most cases.