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加拿大安大略省两家三级医疗中心成年起病皮肌炎患者的特征

Characteristics of Patients With Adult-Onset Dermatomyositis at 2 Tertiary Care Centres in Ontario, Canada.

作者信息

Metko Dea, Bednar Dimitra E, Alkhayal Fares, Pavlova Viktoria, Legault Kimberly, Abu-Hilal Mohannad

机构信息

Michael G. DeGroote School of Medicine, Hamilton, ON, Canada.

Division of Dermatology, University of Toronto, Toronto, ON, Canada.

出版信息

J Cutan Med Surg. 2025 Mar-Apr;29(2):124-130. doi: 10.1177/12034754241301409. Epub 2024 Nov 26.

Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by progressive muscle weakness and distinctive cutaneous findings. The exact incidence and prevalence of DM in the general population is largely unknown, and data on demographic and clinical features in patients in Canada are lacking. This study aimed to comprehensively evaluate the patients with DM in Southwestern Ontario, Canada. A retrospective chart review was conducted for patients with adult-onset DM at 2 tertiary care centres in Ontario, Canada, over a 13 year period. One hundred fourteen patients were included. The mean age was 59, and 80% of patients were female. Skin and muscle involvement coincided in 63% of patients, while skin predated muscle involvement in 11%. Most common characteristic skin manifestations included Gottron's papules (69%), Heliotrope sign (66%), V sign (61%), and Shawl sign (56%). Among the 114 patients, 88 (77%) were myopathic while 26/114 (23%) were clinically amyopathic dermatomyositis. In the myopathic type, upper extremity weakness, lower extremity weakness, and dysphagia were present in 87%, 81%, and 29% of patients, respectively. Elevated creatine kinase, C-reactive protein, lactate dehydrogenase, and erythrocyte sedimentation rate were found in 66%, 35%, 33%, and 32%, respectively. Lung disease was reported in 39%, particularly interstitial lung disease. Other associated features included malignancy, cardiovascular disease, and less commonly gastrointestinal disease. DM is a multifaceted autoimmune disease with distinct cutaneous and muscular findings. Our study results align with the growing body of evidence supporting DM as a complex systemic disease with the potential involvement of other organs such as the pulmonary, cardiovascular, and gastrointestinal systems.

摘要

皮肌炎(DM)是一种特发性炎性肌病,其特征为进行性肌无力和独特的皮肤表现。普通人群中DM的确切发病率和患病率很大程度上尚不清楚,且缺乏加拿大患者的人口统计学和临床特征数据。本研究旨在全面评估加拿大安大略省西南部的DM患者。对加拿大安大略省2个三级医疗中心13年间成年起病的DM患者进行了回顾性病历审查。纳入了114例患者。平均年龄为59岁,80%的患者为女性。63%的患者皮肤和肌肉同时受累,而11%的患者皮肤受累先于肌肉。最常见的特征性皮肤表现包括Gottron丘疹(69%)、向阳性皮疹(66%)、V字征(61%)和披肩征(56%)。在这114例患者中,88例(77%)为肌病型,而26/114例(23%)为临床无肌病性皮肌炎。在肌病型中,分别有87%、81%和29%的患者出现上肢无力、下肢无力和吞咽困难。分别有66%、35%、33%和32%的患者肌酸激酶、C反应蛋白、乳酸脱氢酶和红细胞沉降率升高。39%的患者报告有肺部疾病,尤其是间质性肺病。其他相关特征包括恶性肿瘤、心血管疾病,较少见的还有胃肠道疾病。DM是一种具有独特皮肤和肌肉表现的多方面自身免疫性疾病。我们的研究结果与越来越多的证据一致,这些证据支持DM是一种复杂的全身性疾病,可能累及肺、心血管和胃肠道等其他器官。

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本文引用的文献

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Risk factors for malignancy in adult-onset dermatomyositis.成人起病型皮肌炎的恶性肿瘤危险因素。
J Eur Acad Dermatol Venereol. 2024 Jan;38(1):e122-e124. doi: 10.1111/jdv.19478. Epub 2023 Sep 12.
2
Distinctive cutaneous features of dermatomyositis in Black adults: A case series.成年黑人皮肌炎的独特皮肤特征:病例系列
JAAD Case Rep. 2023 May 22;37:106-109. doi: 10.1016/j.jdcr.2023.05.019. eCollection 2023 Jul.

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