抗合成代谢酶综合征肺部表现回顾。

IF 2.3 3区 医学 Q2 CRITICAL CARE MEDICINE Seminars in respiratory and critical care medicine Pub Date : 2024-06-01 Epub Date: 2024-05-06 DOI:10.1055/s-0044-1785536
Mohammad I Ghanbar, Sonye K Danoff
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引用次数: 0

摘要

抗合成代谢酶综合征(ASyS)是目前公认的特发性炎症性肌病中的一种。该病最初是在肌炎、关节炎和间质性肺病(ILD)三联症患者中出现,但其表现多种多样。ASyS 患者的常见症状还包括雷诺现象、机械手和发热。虽然该病与多发性肌炎和皮肌炎有明显的重叠,但其主要区别在于是否存在抗异烟酸酶抗体(ASA)。目前已发现多达 10 种 ASA 与 ASyS 的表现相关,每种 ASA 的表现可能与其他 ASyS 略有不同。尽管提出了三种分类标准来帮助诊断,但患者表现的异质性仍带来了挑战。在 ASyS 患者中,ILD 是一个重要的负担,有时会单独出现。值得注意的是,ILD 通常也是 ASyS 的首发症状,这就要求肺科医生保持警惕,以做出准确诊断。本文将全面回顾 ASyS 的各个方面,包括疾病的表现、诊断、管理和临床过程,重点关注其肺部表现。
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Review of Pulmonary Manifestations in Antisynthetase Syndrome.

Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations.

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来源期刊
CiteScore
6.10
自引率
0.00%
发文量
87
审稿时长
6-12 weeks
期刊介绍: The journal focuses on new diagnostic and therapeutic procedures, laboratory studies, genetic breakthroughs, pathology, clinical features and management as related to such areas as asthma and other lung diseases, critical care management, cystic fibrosis, lung and heart transplantation, pulmonary pathogens, and pleural disease as well as many other related disorders.The journal focuses on new diagnostic and therapeutic procedures, laboratory studies, genetic breakthroughs, pathology, clinical features and management as related to such areas as asthma and other lung diseases, critical care management, cystic fibrosis, lung and heart transplantation, pulmonary pathogens, and pleural disease as well as many other related disorders.
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