病例报告:中叶综合征:一种罕见的表现,发生在带有多层炎的酸性粒状炎中
Beatrice Maranini1, Ippolito Guzzinati2, Gian Luca Casoni2
1Division of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
Frontiers in immunology
|August 28, 2023
概括
中叶综合征 (MLS) 在异炎 (EGPA) 的eosinophilic granulomatosis中被成功用光纤支气管镜治疗. 这一案例凸显了在EGPA中识别MLS对于有效的患者管理的重要性.
科学领域:
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
- 内部医学 内部医学
背景情况:
- 抗中性细胞质抗体 (ANCA) 相关的血管炎 (AAV) 包括死性炎症和循环ANCA的疾病.
- 带有多炎的埃索诺菲尔颗粒瘤 (EGPA) 是一种AAV亚型,其特点是埃索诺菲利,神经病变和器官特异性参与.
- 中叶叶综合征 (MLS) 涉及右侧中叶的反复复发的,通常是喘的并发症.
研究的目的:
- 在EGPA患者中描述一个独特的MLS病例.
- 为了说明EGPA的背景下MLS的诊断和治疗方法.
- 强调在EGPA中承认MLS对于预后和管理的重要性.
主要方法:
- 一个51岁的女性EGPA患者的病例介绍,患有MLS.
- 诊断程序包括胸部CT,光纤支气管镜 (FOB) 和支气管活检.
- 治疗包括保守的管理,然后是内镜呼吸道阻塞管理.
主要成果:
- 患者呈现白细胞症和急性阶段蛋白质升高,没有典型的呼吸道症状.
- 胸部CT显示了完整的中叶脱氧和支气管阻塞.
- FOB证实了支气管狭窄,内镜治疗导致了阿特莱克塔斯症的解决.
结论:
- 这是EGPA的MLS在FOB成功治疗的第一份报告.
- 在EGPA中识别MLS至关重要,因为与其他肺部AAV表现相比,它可能需要不同的管理策略.
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