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皮肌炎:对巴西圣保罗市临床医院(HCFMUSP)调查的109例患者的分析。

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil.

作者信息

Ortigosa Luciena Cegatto Martins, Reis Vitor Manuel Silva dos

机构信息

Universidade de São Paulo, São Paulo, SP, Brazil.

出版信息

An Bras Dermatol. 2014 Sep-Oct;89(5):719-27. doi: 10.1590/abd1806-4841.20143422.

Abstract

BACKGROUND

Dermatomyositis affects striated muscles, skin and other organs.

OBJECTIVE

To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature.

METHODS

Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases.

RESULTS

Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%.

CONCLUSION

in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small.

摘要

背景

皮肌炎会影响横纹肌、皮肤和其他器官。

目的

对1992年1月至2002年12月期间的该疾病进行特征描述,评估其分类、皮肤和全身表现,以及与文献相比的实验室检查结果、治疗和预后情况。

方法

从109例患者的病历中获取数据,这些患者被分为五组:23例青少年皮肌炎;59例原发性特发性皮肌炎;6例无肌病性皮肌炎;7例与肿瘤相关的皮肌炎;14例与其他结缔组织病相关的皮肌炎。

结果

60例患者被分类为“确诊”;33例为“可能”;4例为“疑似”;12例为无肌病性。诊断时的平均年龄为36岁。所有患者均有皮肤表现;最常见的症状是近端肌无力;最常见的肺部疾病是间质性肺病,胃炎是最常见的消化系统表现。6.42%的病例记录有肿瘤。大多数病例中乳酸脱氢酶是最常升高的肌肉酶。68例患者进行了皮肤活检;53例进行了肌肉活检;58例进行了神经肌电图检查。最常用的治疗方法是皮质激素治疗,死亡率为14.7%。

结论

在这个样本中,该疾病在较年轻个体中出现,女性更为常见,与癌症的关联较小。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6946/4155949/a229da18f535/abd-89-05-0719-g01.jpg

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