关键词: Dermatomyositis idiopathic inflammatory myopathy malignancy paraneoplastic syndrome Dermatomyositis idiopathic inflammatory myopathy malignancy paraneoplastic syndrome

来  源:   DOI:10.12865/CHSJ.47.03.07   PDF(Pubmed)

Abstract:
The idiopathic inflammatory myopathies (IIM) are a group of heterogeneous systemic diseases which include as main subtypes: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). The key feature of IIMs is the muscle weakness, accompanied by a characteristic skin rash in DM patients. The overall risk for malignancy in IIM is higher compared to the age-and sex-matched general population. Most epidemiologic studies have included only PM and DM patients and reported consistently higher rates of malignancy in DM. Most common types of cancer in DM are adenocarcinoma of the lung, ovary or gastrointestinal tract, melanoma and non-Hodgkins lymphoma. The highest risk for malignancy is seen in the first year after DM diagnosis. Multiple disease features have been linked to the development of cancer in DM. These include: older age, male sex, skin necrosis, Gottron sign, heliotrope rash, dysphagia, low complement C4, lymphocytosis, poor response to corticosteroids and rapid disease progression. Our study included 23 patients with DM, divided into two groups based on the association of malignancy, in order to compare clinical and demographic features, laboratory markers and analyze characteristic of cancer development.
摘要:
特发性炎性肌病(IIM)是一组异质性的全身性疾病,主要包括:多发性肌炎(PM),皮肌炎(DM)和包涵体肌炎(IBM)。IIM的主要特征是肌肉无力,DM患者伴有特征性皮疹。与年龄和性别匹配的普通人群相比,IIM中恶性肿瘤的总体风险更高。大多数流行病学研究仅包括PM和DM患者,并且报告了DM中恶性肿瘤的发生率一直较高。DM中最常见的癌症类型是肺腺癌,卵巢或胃肠道,黑色素瘤和非霍奇金淋巴瘤。恶性肿瘤的最高风险是在DM诊断后的第一年。多种疾病特征与DM中癌症的发展有关。这些包括:年龄较大,男性,皮肤坏死,Gottron标志,天草性皮疹,吞咽困难,低补体C4,淋巴细胞增多,对皮质类固醇的反应差和快速的疾病进展。我们的研究包括23名DM患者,根据恶性肿瘤的关系分为两组,为了比较临床和人口统计学特征,实验室标记物和分析癌症发展的特征。
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