罕见的表皮质粒细胞瘤:诊断挑战和治疗影响
Majd Werda1, Ahmed Ben Akacha1, Salim Amouri2
1ENT - Head and Neck Department, Habib Bourguiba University Hospital, Sfax, Tunisia.
Ear, nose, & throat journal
|May 21, 2025
概括
本案例研究详细介绍了一例罕见的多炎 (GPA) 带来的表皮质粒症,该病例发生在患有慢性呼吸道阻塞的女性身上. 用皮质类固醇和修复剂治疗导致症状迅速消失和愈合,强调早期诊断和监测.
科学领域:
- 类风湿病学 类风湿病学
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
- 病理学 病理学 病理学
背景情况:
- 带有多炎 (GPA) 的粒状瘤炎是一种罕见的自身免疫性血管炎,可以影响各种器官.
- 在GPA中,孤立的表皮节干扰是非常罕见的,通常会出现上呼吸道阻塞的非特异性症状.
研究的目的:
- 报告一种罕见的单独表皮质GPA病例.
- 突出诊断挑战和成功的治疗策略,为这种罕见的表现.
- 强调在耐火性上呼吸道阻塞中考虑GPA的重要性.
主要方法:
- 一个72岁妇女的病例介绍,她患有慢性消化不良和呼吸不良.
- 诊断工作包括喉腔镜检查,CT扫描,组织病理学和蛋白酶3抗中性粒细胞抗体 (PR3-ANCA) 血清学测试.
- 治疗涉及高剂量的皮质类固醇和修复剂,随后是维持免疫抑制.
主要成果:
- 组织病理学证实了缩性颗粒瘤,血管炎和多核巨细胞,确立了GPA的诊断.
- 患者在治疗开始后2周内实现了快速症状缓解.
- 在1个月的随访后,观察到粘膜完全愈合,在18个月内没有复发.
结论:
- 在慢性上呼吸道阻塞不响应标准疗法的情况下,应考虑隔离的表皮节GPA.
- PR3-ANCA血清学和活检证明的组织病理学对于准确的诊断至关重要.
- 基于rituximab的疗法对喉GPA有效,但由于复发的可能性,需要长期监测.
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