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机械工手预示着一名抗MDA5阳性青少年皮肌炎印度青少年的病情复发。

Mechanic's Hand Heralding Relapse in an Indian Adolescent with Anti-MDA5 Positive Juvenile Dermatomyositis.

作者信息

Ganatra Keya, Aggarwal Rohit, Gupta Latika

机构信息

Seth Gordhandas Sunderdas Medical College & King Edward Memorial Hospital, Mumbai, India.

Department of Medicine, University of Pittsburgh, Pittsburgh, PA, United States.

出版信息

Mediterr J Rheumatol. 2022 Jun 30;33(2):268-270. doi: 10.31138/mjr.33.2.268. eCollection 2022 Jun.

Abstract

Anti-MDA5 antibodies characterise a distinct phenotype of dermatomyositis in adults as well as children, with ethnic disparity in clinical presentation and severity. They often present as a diagnostic conundrum with rash, ulceration, and polyarthritis, but minimal muscle disease. Mechanic's hands are typically associated with anti-synthetase syndrome, but their presence in anti-MDA5 antibody positive patients, although reported, is not well known. We present the case of a boy in whom mechanic's hand heralded a relapse of juvenile dermatomyositis which was suspected based on remotely assessed patient-reported outcome measures on teleconsultation. This report suggests that mechanic's hands should also prompt testing for myositis antibodies including anti-MDA5 in Indian children with JDM. Diligent awareness of the condition, and timely use of patient reported outcome measures of muscle power and skin assessment may guide management while delivering remote care in challenging situations such as a global pandemic.

摘要

抗MDA5抗体在成人和儿童皮肌炎中表现出独特的表型,在临床表现和严重程度上存在种族差异。它们常表现为伴有皮疹、溃疡和多关节炎但肌肉疾病轻微的诊断难题。技工手通常与抗合成酶综合征相关,尽管有报道称抗MDA5抗体阳性患者中也有技工手,但对此并不十分了解。我们报告了一名男孩的病例,其技工手预示着青少年皮肌炎复发,这是根据远程会诊中通过远程评估的患者报告结局指标怀疑的。本报告表明,在印度患有青少年皮肌炎的儿童中,技工手也应促使检测包括抗MDA5在内的肌炎抗体。在诸如全球大流行这样具有挑战性的情况下提供远程护理时,对该病的充分认识以及及时使用患者报告的肌肉力量和皮肤评估结局指标可能会指导治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3ed4/9450195/5c47bd24e0f3/MJR-33-2-268-g001.jpg

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