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与干燥综合征、系统性红斑狼疮和类风湿关节炎相关的PL-7抗合成酶综合征

PL-7 Antisynthetase Syndrome in Association with Sjögren's, Systemic Lupus Erythematosus, and Rheumatoid Arthritis.

作者信息

Jawaid Mehrin, Ross Yael, Kamran Mohammad

机构信息

WellStar Kennestone Regional Medical Center, Marietta, GA 30060, USA.

出版信息

Case Rep Rheumatol. 2020 Feb 13;2020:4736476. doi: 10.1155/2020/4736476. eCollection 2020.

Abstract

We present a rare case of PL-7 antisynthetase syndrome (ASS) in association with Sjögren's, systemic lupus erythematosus (SLE), and seropositive rheumatoid arthritis (RA). Initially, the patient was diagnosed with Sjögren's followed by Sjögren's/SLE overlap and then Sjögren's/SLE/RA overlap. She was eventually diagnosed with Sjögren's/SLE/RA overlap with PL-7 ASS with interstitial lung disease (ILD). ILD was discovered after complaints of pleuritic chest pain with subsequent workup with coronary computed tomography (CT) revealing pulmonary fibrosis. This case demonstrates the ambiguity with which symptoms of ASS can present; given the high respiratory morbidity and mortality of ASS especially in non-Jo-1 patients, those who present with Raynaud's, myositis, or joint pain, whether together or in isolation, should be assessed for presence of additional features of ASS and potentially undergo testing for ASS antibodies if appropriate.

摘要

我们报告了一例罕见的PL-7抗合成酶综合征(ASS),该病例同时合并干燥综合征、系统性红斑狼疮(SLE)和血清阳性类风湿关节炎(RA)。最初,该患者被诊断为干燥综合征,随后诊断为干燥综合征/系统性红斑狼疮重叠综合征,再后来诊断为干燥综合征/系统性红斑狼疮/类风湿关节炎重叠综合征。最终,她被诊断为干燥综合征/系统性红斑狼疮/类风湿关节炎重叠综合征合并PL-7 ASS及间质性肺疾病(ILD)。患者因胸膜炎性胸痛就诊,随后进行冠状动脉计算机断层扫描(CT)检查发现肺纤维化,从而确诊为ILD。该病例表明ASS症状可能具有模糊性;鉴于ASS尤其是非Jo-1型患者的呼吸道发病率和死亡率较高,对于出现雷诺现象、肌炎或关节疼痛的患者,无论这些症状是同时出现还是单独出现,都应评估是否存在ASS的其他特征,并在适当情况下进行ASS抗体检测。

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