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意大利一项针对1152例重症肌无力患者的多中心随访研究。

A multicentre follow-up study of 1152 patients with myasthenia gravis in Italy.

作者信息

Mantegazza R, Beghi E, Pareyson D, Antozzi C, Peluchetti D, Sghirlanzoni A, Cosi V, Lombardi M, Piccolo G, Tonali P

机构信息

Neurological Institute C. Besta, Milan, Italy.

出版信息

J Neurol. 1990 Oct;237(6):339-44. doi: 10.1007/BF00315656.

DOI:10.1007/BF00315656
PMID:2277266
Abstract

A multicentre retrospective study was carried out on the characteristics and course of myasthenia gravis (MG) in Italy. Data from 1152 patients, fairly representative of the myasthenic population seeking medical advice, were analysed for diagnostic criteria, clinical aspects and therapeutic approaches. Mean follow-up was 4.9 years. The disease was correctly diagnosed within 2 years of the onset in 80% of cases. Onset of symptoms peaked in the second and third decade in females and fell between 20 and 59 years in males. At first observation 87% of the patients had generalized MG. Maximal worsening was observed within 3 years in 77% of patients. At the last follow-up, 35% of cases were symptom-free (pharmacological remission 24%, remission without treatment 11%). The more severe the disease at the first observation and at the maximal worsening of symptoms, the lower was the proportion of remissions. Steroids were given in 54% and immunosuppressants in 18%. Thymectomy was performed in 72%, mostly in women, younger than age 40, and with generalized MG. Thymectomy seemed to improve the course of the disease, mostly in patients operated on shortly after diagnosis and those with generalized mild-to-moderate disease and with a normally involuted thymus. MG was lethal in 4% of patients, principally men, older than 40, in grade 3 or worse at first observation, with a short history of disease, and with thymona.

摘要

对意大利重症肌无力(MG)的特征及病程开展了一项多中心回顾性研究。分析了1152例患者的数据,这些数据能较好地代表寻求医疗建议的肌无力人群,内容涉及诊断标准、临床症状及治疗方法。平均随访时间为4.9年。80%的病例在发病2年内得到正确诊断。女性症状发作高峰期在第二和第三个十年,男性则在20至59岁之间。初次观察时,87%的患者患有全身型MG。77%的患者在3年内病情达到最严重程度。在最后一次随访时,35%的病例无症状(药物缓解24%,未治疗缓解11%)。初次观察及症状最严重时病情越严重,缓解比例越低。54%的患者使用了类固醇,18%使用了免疫抑制剂。72%的患者接受了胸腺切除术,其中大多数是年龄小于40岁、患有全身型MG的女性。胸腺切除术似乎改善了疾病进程,主要是对诊断后不久接受手术的患者以及患有全身型轻至中度疾病且胸腺正常退化的患者。4%的患者死于MG,主要是年龄大于40岁、初次观察时病情为3级或更严重、病程短且患有胸腺瘤的男性。

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