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商业检测法确定的伴黑色素瘤分化相关蛋白 5 自身抗体阳性的成人皮肌炎患者的临床和组织病理学特征:一项回顾性、单机构比较队列研究。

Clinical and histopathological features of adult patients with dermatomyositis and melanoma differentiation associated-5 autoantibody seropositivity status, as determined by commercially available testing: a retrospective, single-institution comparative cohort study.

机构信息

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

Department of Dermatology, Florida State University, Tallahassee, FL, USA.

出版信息

Clin Exp Dermatol. 2022 Feb;47(2):282-288. doi: 10.1111/ced.14870. Epub 2021 Oct 5.

Abstract

BACKGROUND

Although melanoma differentiation associated (MDA)-5 autoantibodies have been widely explored in dermatomyositis (DM), most studies have relied on MDA-5 autoantibody testing performed in research settings, rather than the now-available commercial laboratory tests.

AIM

To characterize the clinical and histopathological data in patients with DM and circulating MDA-5 autoantibodies, as defined by commercially available testing.

METHODS

This was a retrospective review of patients with DM who underwent MDA-5 antibody testing. All available skin biopsy slides were reviewed.

RESULTS

Cutaneous features more prevalent in MDA-5-positive DM included Raynaud phenomenon (RP) (P < 0.001), cutaneous ulcerations (P = 0.01), mechanic hands (P < 0.02), palmar papules (P < 0.01), oral ulcers (P = 0.024) and alopecia (P = 0.03). Joint and pulmonary involvement were more common in patients with MDA-5-positive DM (both P < 0.001) as was dysphagia (P < 0.01). Myopathy (P = 0.4) and malignancy (P = 0.34) were not statistically different between the cohorts. Vasculopathy was more common in MDA-5-positive DM (P < 0.01), while spongiosis was less common (P < 0.02).

CONCLUSION

This study not only confirms some known associations between disease manifestations and MDA-5 autoantibody status, as determined by commercially available tests, but also identifies new associations, including RP and dysphagia.

摘要

背景

尽管黑色素瘤分化相关(MDA)-5 自身抗体已在皮肌炎(DM)中广泛研究,但大多数研究依赖于在研究环境中进行的 MDA-5 自身抗体检测,而不是现在可用的商业实验室检测。

目的

描述通过商业上可用的检测方法定义的具有 DM 和循环 MDA-5 自身抗体的患者的临床和组织病理学数据。

方法

这是对接受 MDA-5 抗体检测的 DM 患者的回顾性研究。回顾了所有可用的皮肤活检切片。

结果

在 MDA-5 阳性 DM 中更常见的皮肤特征包括雷诺现象(RP)(P<0.001)、皮肤溃疡(P=0.01)、机械手(P<0.02)、手掌丘疹(P<0.01)、口腔溃疡(P=0.024)和脱发(P=0.03)。MDA-5 阳性 DM 患者的关节和肺部受累更常见(均 P<0.001),吞咽困难也更常见(P<0.01)。肌病(P=0.4)和恶性肿瘤(P=0.34)在两个队列之间无统计学差异。MDA-5 阳性 DM 中血管病变更常见(P<0.01),而海绵状变性更少见(P<0.02)。

结论

本研究不仅证实了一些已知的疾病表现与 MDA-5 自身抗体状态之间的关联,这些关联是通过商业上可用的检测方法确定的,而且还确定了一些新的关联,包括 RP 和吞咽困难。

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