结缔组织疾病中的内支气管阻塞:一种不常见但危及生命的并发症:两个病例报告
Rushab Shah1, Lisa Lim1, Mandana Nikpour2,3
1Department of Rheumatology, St Vincent's Hospital (Melbourne), 41 Victoria Parade, Fitzroy, VIC, 3065, Australia.
Journal of medical case reports
|August 1, 2023
概括
严重的支气管内膜阻塞是一种罕见的,伴有多胞膜炎和复发性多胞膜炎的颗粒瘤症并发症. 包括手术和免疫抑制在内的多学科护理对于管理这些具有挑战性的呼吸道疾病至关重要.
科学领域:
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
- 结合组织疾病 结合组织疾病
背景情况:
- 带有多炎 (GPA) 的颗粒瘤和复发性多炎 (RP) 是罕见的,多系统性结缔组织疾病.
- 内分支障碍是一种严重的,尽管不经常报告的,GPA和RP的并发症.
研究的目的:
- 在GPA和RP中呈现两例严重的支骨内膜阻塞病例.
- 讨论与这些罕见的呼吸道并发症相关的诊断和治疗挑战.
主要方法:
- 案例1:一个患有复发性多合体炎的患者接受了气管切除和重建,并用免疫抑制疗法治疗支气管缩和狭窄.
- 案例2:一个患有多管炎的颗粒瘤症的患者接受了为导致气道封闭的内支气管瘤进行手术切除,并启动了免疫抑制.
主要成果:
- 病例1在手术和免疫抑制后实现了呼吸系统的恢复.
- 病例2显示了免疫抑制的初始稳定,但尽管接受了治疗,疾病的进展仍在继续.
结论:
- 结合免疫抑制,支持性护理和选择性外科干预的多学科方法至关重要.
- 结合性组织疾病中的内支气管阻塞可能由于重叠的症状而未得到报道,并且没有建立最佳实践指南.
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