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多发性肌炎和皮肌炎的肺部受累情况。

Pulmonary involvement in polymyositis and in dermatomyositis.

作者信息

Marie I, Hatron P Y, Hachulla E, Wallaert B, Michon-Pasturel U, Devulder B

机构信息

Département de Médecine Interne, Hôpital Claude Huriez, CHU Lille, France.

出版信息

J Rheumatol. 1998 Jul;25(7):1336-43.

PMID:9676766
Abstract

OBJECTIVE

To assess prevalence, characteristics, and predictive factors of pulmonary involvement in polymyositis (PM) and dermatomyositis (DM).

METHODS

The medical records of 55 consecutive patients with PM and DM between 1983 and 1996 were reviewed. The criteria for diagnosis of PM and DM were based upon Bohan and Peter criteria.

RESULTS

Twenty-two patients (40%) developed lung impairment with a mean of 17 months after onset of PM and DM. The 3 main types of pulmonary disorders were interstitial lung disease (41%), ventilatory insufficiency with bronchopneumonia (22.8%), and alveolitis (with pulmonary function tests revealing restrictive pattern, low diffusing capacity, and normal computerized tomography) (36.2%). Both morbidity and mortality rates were as high as 40 and 27%, respectively, in PM and DM patients with lung involvement. Moreover, for the group with pulmonary diseases, serum GOT and ferritin levels were higher (p = 0.01 and p = 0.02) and the presence of anti-Jo-1 antibody and characteristic microangiopathy were more frequent (p < 0.05 and p = 0.0002).

CONCLUSION

This series reveals both high prevalence (40%) and severity of pulmonary involvement in PM and DM. Our findings also suggest that high serum GOT and ferritin levels, presence of anti-Jo-1 antibody and characteristic microangiopathy may have predictive value and should encourage the search for pulmonary dysfunction and interstitial lung disease in patients with PM and DM.

摘要

目的

评估多发性肌炎(PM)和皮肌炎(DM)肺部受累的患病率、特征及预测因素。

方法

回顾了1983年至1996年间连续收治的55例PM和DM患者的病历。PM和DM的诊断标准基于博汉和彼得标准。

结果

22例患者(40%)出现肺部损害,平均在PM和DM发病后17个月。肺部疾病的3种主要类型为间质性肺疾病(41%)、通气功能不全合并支气管肺炎(22.8%)以及肺泡炎(肺功能检查显示限制性模式、低弥散能力且计算机断层扫描正常)(36.2%)。肺部受累的PM和DM患者的发病率和死亡率分别高达40%和27%。此外,对于患有肺部疾病的患者组,血清谷草转氨酶(GOT)和铁蛋白水平更高(p = 0.01和p = 0.02),抗Jo - 1抗体的存在和特征性微血管病更为常见(p < 0.05和p = 0.0002)。

结论

该系列研究揭示了PM和DM肺部受累的高患病率(40%)和严重性。我们的研究结果还表明,高血清GOT和铁蛋白水平、抗Jo - 1抗体的存在以及特征性微血管病可能具有预测价值,应促使对PM和DM患者的肺功能障碍和间质性肺疾病进行筛查。

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