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以肺部受累为主的抗合成酶综合征:一例报告

Antisynthetase Syndrome With Predominant Pulmonary Involvement: A Case Report.

作者信息

Lima Corrêa de Araújo Beatriz, Victor David R, Farias Fontes Heloísa Maria, Caminha Mendes Gomes Rayana Maria, Lima Corrêa de Araújo Leonardo

机构信息

Department of Internal Medicine, Hospital Barão de Lucena, Recife, BRA.

Medical School, Universidade de Pernambuco, Recife, BRA.

出版信息

Cureus. 2023 Aug 23;15(8):e43966. doi: 10.7759/cureus.43966. eCollection 2023 Aug.

DOI:10.7759/cureus.43966
PMID:37746430
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10515292/
Abstract

Antisynthetase syndrome (ASyS) is an autoimmune disease characterized by the presence of aminoacyl-transfer RNA synthetase antibodies. Its clinical presentation is variable and may include interstitial lung disease (ILD), myositis, arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." ILD is more prevalent in this entity when compared to other idiopathic inflammatory myopathies and imparts greater severity to the condition. Here, we report the case of a 42-year-old female patient who sought care for severe ILD and persistent fever. Her diagnosis was made only after the detection of anti-Jo1 autoantibodies. Treatment was refractory to both prednisone monotherapy and cyclophosphamide pulse therapy, requiring the introduction of rituximab. A high degree of clinical suspicion is required to allow early diagnosis of ASyS in patients with pulmonary involvement in the absence of accompanying muscle weakness or other clinical symptoms.

摘要

抗合成酶综合征(ASyS)是一种自身免疫性疾病,其特征是存在氨酰 - 转运RNA合成酶抗体。其临床表现多样,可能包括间质性肺病(ILD)、肌炎、关节炎、发热、雷诺现象和“技工手”。与其他特发性炎性肌病相比,ILD在该疾病中更为普遍,且使病情更为严重。在此,我们报告一例42岁女性患者,她因严重ILD和持续发热前来就诊。仅在检测到抗Jo1自身抗体后才做出诊断。泼尼松单药治疗和环磷酰胺脉冲治疗均无效,需要使用利妥昔单抗。对于肺部受累但无伴随肌肉无力或其他临床症状的患者,需要高度的临床怀疑才能早期诊断ASyS。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/891bb734a1c0/cureus-0015-00000043966-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/1fe228506dbc/cureus-0015-00000043966-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/b40dcc2dd9cc/cureus-0015-00000043966-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/891bb734a1c0/cureus-0015-00000043966-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/1fe228506dbc/cureus-0015-00000043966-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/b40dcc2dd9cc/cureus-0015-00000043966-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a83/10515292/891bb734a1c0/cureus-0015-00000043966-i03.jpg

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本文引用的文献

1
Defining anti-synthetase syndrome: a systematic literature review.抗合成酶综合征的定义:系统文献回顾。
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A narrative review of interstitial lung disease in anti-synthetase syndrome: a clinical approach.抗合成酶综合征中间质性肺病的叙述性综述:临床方法
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Antisynthetase syndrome - much more than just a myopathy.抗合成酶综合征——远不止一种肌病。
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Clinical manifestations and treatment of antisynthetase syndrome.抗合成酶综合征的临床表现和治疗。
Best Pract Res Clin Rheumatol. 2020 Aug;34(4):101503. doi: 10.1016/j.berh.2020.101503. Epub 2020 Apr 11.
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Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course.抗合成酶抗体特异性对抗合成酶综合征临床谱进程的影响。
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Sjögren syndrome.干燥综合征。
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Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome: Results From an International Retrospective Multicenter Study.抗 Jo-1 阳性抗合成酶综合征的临床谱随时间变化:一项国际回顾性多中心研究的结果
Medicine (Baltimore). 2015 Aug;94(32):e1144. doi: 10.1097/MD.0000000000001144.
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An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.欧洲呼吸学会/美国胸科学会官方研究声明:具有自身免疫特征的间质性肺炎。
Eur Respir J. 2015 Oct;46(4):976-87. doi: 10.1183/13993003.00150-2015. Epub 2015 Jul 9.