[与系统性红斑狼相关的缩小肺综合征]
Isabella Perrone1, Lorena Buffarini1, Pablo Szwarstein1
1Unidad Neumotisiología, Centro Universitario de Neumonología, Facultad de Medicina UBA, Buenos Aires, Argentina.
收缩性肺综合征 (SLS) 是一种罕见的系统性红斑狼 (SLE) 症状,影响10.3%的SLE患者. 呼吸不良是常见的,在成像上经常看到食,需要免疫抑制治疗.
科学领域:
- 肺部医学 肺部医学
- 类风湿病学 类风湿病学
- 罕见疾病 罕见疾病
背景情况:
- 缩小肺综合征 (SLS) 是系统性红斑狼 (SLE) 的不常见表现.
- 了解SLE患者的SLS特征对于诊断和管理至关重要.
研究的目的:
- 在SLE患者队列中描述SLS的人口,临床,功能和成像特征.
- 评估在SLE中用于SLS的治疗策略.
主要方法:
- 对SLS患者的SLE临床记录的回顾性审查.
- 2007年至2023年间在风湿病学和肺病学部门的预期随访.
主要成果:
- 在107名SLE患者中,SLS被诊断为10.3% (81.8%为女性,中位数年龄为30岁).
- 呼吸障碍 (72%) 是最常见的症状;27.3%是无症状的.
- 肺功能显示中度限制与轻度DLCO恶化. 最常见的HRCT发现是Atelectasis (54.5%).这是最常见的HRCT发现.
结论:
- 在SLE患者中,SLS的患病率高于之前报告的.
- 喘息不良和消化不良是SLS的关键临床和成像发现.
- 所有患者都接受了免疫抑制疗法,主要是与其他药物结合的全身皮质类固醇.
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