反合成酶综合征伪装成复发性肺炎
1Medicine, Temple University Hospital, Philadelphia, Pennsylvania, USA kau.lio@tuhs.temple.edu.
BMJ case reports
|June 5, 2023
概括
抗合成酶综合征 (ASS) 可以模仿肺炎,导致延迟诊断ASS相关的间歇性肺病 (ASS-ILD). 早期的自身抗体检测对于识别这种罕见的自身免疫性疾病至关重要.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 免疫学 免疫学 免疫学
背景情况:
- 抗合成酶综合征 (ASS) 是一种罕见的自身免疫性肌肉病,具有多样化的临床表现.
- 与ASS相关的间歇性肺病 (ASS-ILD) 可以呈现急性,模仿常见的呼吸道感染,如肺炎.
- 间歇性肺病 (ILD) 可能是ASS的唯一表现特征,使诊断复杂化.
研究的目的:
- 要突出与抗合成酶综合征相关的间歇性肺病的诊断挑战.
- 强调在耐火性肺炎和肌酸酶升高的患者中考虑ASS的重要性.
- 为了强调自身抗体测试在不分化自身免疫疾病中的实用性.
主要方法:
- 一个50多岁的女性病例报告,患有复发性呼吸不良和疑似多焦点肺炎.
- 诊断评估包括肌酸激酶水平,胸部CT,支气管支气管洗与支气管支气管支气管扫描以及肌炎面板.
- 治疗包括静脉注射免疫球蛋白和甲基prednisolone.
主要成果:
- 患者出现了模仿肺炎的症状,但抗生素治疗无效.
- 肌酸激酶 (3258 U/L) 的升高以及对磨砂玻璃不透明度的CT发现表明间歇性肺部疾病.
- 阳性抗Jo-1抗体证实了ASS-ILD的诊断. 这是一个很好的结果.
- 治疗导致显著的临床改善,包括 hypoxemia 的解决方案.
结论:
- 由于其非特异性和可变的表现,ASS-ILD可能被误诊,通常类似于常见的急性肺部疾病.
- 早期怀疑和特定的自身抗体检测对于准确诊断和及时管理ASS-ILD至关重要.
- 这一案例强调了在患有不明原因呼吸道症状和全身炎症标志物的患者中需要广泛的差异诊断.
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