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技工手;它是抗合成酶综合征疾病复发的前驱症状吗?一例病例报告。

Mechanic's hand; is it a prodromic sign of disease relapse of anti-synthetase syndrome; a case report.

作者信息

Rosa C T, Thilakarathne A S, Senevirathne L A, Wijeyeratna A, Munidasa D

机构信息

Rheumatology and Rehabilitation Hospital, Ragama, Sri Lanka.

出版信息

BMC Rheumatol. 2021 Jul 26;5(1):25. doi: 10.1186/s41927-021-00195-2.

Abstract

BACKGROUND

Anti-synthetase syndrome is the collection of myositis and/or interstitial lung disease with the presence of various antibodies directed against an aminoacyl transfer RNA synthetase. Anti Jo - 1 antibody is the commonest of these antibodies and its presence is characteristically associated with the dermatological manifestation of mechanic's hands. However, in the absence of other features, whether the presence of mechanic's hands could be considered as a prodromic sign of disease relapse is not proven. We would like to present a patient who developed mechanic's hands and subsequently went on to have recurrence in her myositis.

CASE PRESENTATION

A 45-year-old female initially presented with a progressive proximal muscle weakness. Her muscle enzymes were elevated, EMG and biopsy were also in keeping with an inflammatory myositis. Subsequently she was found to have an interstitial lung disease with a non-specific interstitial pneumonitis pattern radiologically. Her anti Jo-1 was positive. However, she did not have any dermatological manifestations at the time. With immunosuppressive therapy she achieved remission which lasted for about 2 years. Then she developed fissuring and cracking of the palms and fingers suggestive of mechanic's hands without any muscle pain, weakness and elevation of muscle enzymes. A few months later she did develop muscle pain, weakness and elevation of muscle enzymes heralding a disease relapse.

CONCLUSION

The presence of mechanic's hands without other features should be considered as a prodromic sign of disease relapse.

摘要

背景

抗合成酶综合征是指伴有多种针对氨酰基转移RNA合成酶抗体的肌炎和/或间质性肺疾病。抗Jo-1抗体是这些抗体中最常见的,其存在通常与技工手的皮肤表现相关。然而,在没有其他特征的情况下,技工手的出现是否可被视为疾病复发的前驱症状尚未得到证实。我们想介绍一位出现技工手,随后肌炎复发的患者。

病例介绍

一名45岁女性最初表现为进行性近端肌无力。她的肌酶升高,肌电图和活检也符合炎性肌炎。随后,她被发现患有间质性肺疾病,放射学表现为非特异性间质性肺炎模式。她的抗Jo-1抗体呈阳性。然而,当时她没有任何皮肤表现。经过免疫抑制治疗,她达到了缓解,持续了约2年。然后她出现了手掌和手指的皲裂和开裂,提示为技工手,没有任何肌肉疼痛、无力和肌酶升高。几个月后,她确实出现了肌肉疼痛、无力和肌酶升高,预示着疾病复发。

结论

没有其他特征的技工手的出现应被视为疾病复发的前驱症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e48/8311943/6f1dfadb60bd/41927_2021_195_Fig1_HTML.jpg

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