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[成人多肌炎和皮肌炎。突尼斯多中心研究]

[Polymyositis and dermatomyositis in adults. Tunisian multicentre study].

作者信息

Toumi S, Ghnaya H, Braham A, Harrabi I, Laouani-Kechrid C

机构信息

Service de médecine interne, CHU Sahloul, 4054, route de ceinture, Sousse, Tunisia.

出版信息

Rev Med Interne. 2009 Sep;30(9):747-53. doi: 10.1016/j.revmed.2009.03.356. Epub 2009 Aug 14.

Abstract

INTRODUCTION

To study the clinical features and the outcome of polymyositis (PM) and dermatomyositis (DM) in Tunisia.

METHODS

Retrospective multicentre study conducted by the Tunisian Society of Internal Medicine. Adult patients with confirmed and probable PM and DM, according to the Bohan and Peter's criteria, were included in the study and followed-up between 1987 and 2005.

RESULTS

Twenty cases of PM and 50 cases of DM were included. Female/male sex-ratio was 2.5. The mean age at diagnosis was 40.7 years. Muscle weakness concerned girdle muscles in 94.3% and pharyngeal muscles in 42.8% of the patients. Interstitial lung disease and heart involvement occurred respectively in 35.7 and 20%. Eyelid erythema, peri-ungual erythema and Gottron's sign occurred in respectively 86, 50 and 36% of the cases of DM. Malignant disease was found in 12.8% of the patients (mainly gynecological and nasopharyngeal cancers). All patients received high dose corticosteroids with additional immunosuppressive drugs in 40% of the cases. The outcome was stabilization or partial improvement in 71.5% of the cases. Death occurred in 20% and was caused by respiratory failure, sepsis and cardiac failure.

CONCLUSION

The particularities of PM and DM in Tunisia are the preponderance of DM, early onset of the disease and high frequency of the nasopharyngeal cancer.

摘要

引言

研究突尼斯多发性肌炎(PM)和皮肌炎(DM)的临床特征及预后。

方法

突尼斯内科协会开展的回顾性多中心研究。根据博汉和彼得标准确诊及疑似的成年PM和DM患者纳入研究,并于1987年至2005年进行随访。

结果

纳入20例PM和50例DM患者。女性/男性性别比为2.5。诊断时的平均年龄为40.7岁。94.3%的患者肌肉无力累及带肌,42.8%累及咽肌。间质性肺病和心脏受累分别发生在35.7%和20%的患者中。DM患者中分别有86%、50%和36%出现眼睑红斑、甲周红斑和Gottron征。12.8%的患者发现恶性疾病(主要为妇科和鼻咽癌)。所有患者均接受大剂量皮质类固醇治疗,40%的病例加用免疫抑制药物。71.5%的病例病情稳定或部分改善。20%的患者死亡,死因是呼吸衰竭、败血症和心力衰竭。

结论

突尼斯PM和DM的特点是DM占优势、疾病发病早以及鼻咽癌发病率高。

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