抗合成酶综合征：双侧磨玻璃影的罕见且具挑战性的诊断——附文献复习的病例报告。
Anti-synthetase syndrome: a rare and challenging diagnosis for bilateral ground-glass opacities-a case report with literature review.
机构信息
Department of Internal Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, 07753, USA.
Department of Pulmonology and Critical Care, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA.
出版信息
BMC Pulm Med. 2021 Jan 6;21(1):11. doi: 10.1186/s12890-020-01388-0.
BACKGROUND
Anti-synthetase syndrome (ASS) is an uncommon immune-mediated entity characterized by myositis, interstitial lung disease (ILD), non-erosive arthritis, and less common features such as fever, Raynaud's phenomenon, and skin changes in association with anti-aminoacyl-transfer-RNA antibodies, most commonly anti-Jo-1 antibodies.
CASE PRESENTATION
We present a challenging and rare case of ASS-associated ILD presenting with unexplained respiratory symptoms and bilateral infiltrates on chest imaging during the COVID-19 pandemic. High clinical suspicion for ASS with early appropriate therapy with corticosteroids and immunosuppressive agents led to marked clinical improvement.
CONCLUSION
High index of suspicion for ASS is mandated in patients with unexplained ILD. A comprehensive autoimmune work-up is important as an early treatment with corticosteroids with or without immunomodulators improves patient outcomes and survival in an otherwise poor prognostic disease.
背景
抗合成酶综合征（ASS）是一种罕见的免疫介导性疾病，其特征为肌炎、间质性肺疾病（ILD）、非侵蚀性关节炎以及较少见的发热、雷诺现象和皮肤改变等特征，与抗氨酰基-tRNA 抗体相关，最常见的是抗-Jo-1 抗体。
病例介绍
我们报告了一个具有挑战性和罕见的抗合成酶综合征相关的间质性肺疾病的病例，该病例在 COVID-19 大流行期间出现了原因不明的呼吸道症状和双侧浸润性肺部影像。高临床疑诊为抗合成酶综合征，早期应用皮质类固醇和免疫抑制剂进行适当治疗，导致了显著的临床改善。
结论
对于原因不明的 ILD 患者，需要高度怀疑 ASS。全面的自身免疫性疾病检查非常重要，因为早期使用皮质类固醇加或不加免疫调节剂治疗可以改善患者的预后和生存，否则该疾病的预后较差。
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