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在患有复发性多合体炎和性结肠炎的患者中表性纤维生化
Chakorn Lapanan1, Krittika Teerapuncharoen2, Suwimon Wonglaksanapimon3
1Division of Respiratory Disease and Tuberculosis, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand chakorn.lap@mahidol.ac.th.
BMJ case reports
|October 10, 2025
概括
这项案例研究表明,多发性膜纤维生结症 (PPFE) 和性结肠炎之间可能存在联系. 在炎症性肠病患者中早期识别这种罕见的肺病对于管理至关重要.
科学领域:
- 肺部病理学 肺部病理学
- 胃肠病学 胃肠病学
- 类风湿病学 类风湿病学
背景情况:
- 炎症性肠病 (IBD),如性结肠炎 (UC),可以呈现出肠外表现.
- 间歇性肺部疾病 (ILD) 是与IBD相关的罕见但严重的肺部并发症.
- 斑关节性纤维生化 (PPFE) 是一种罕见的异常性ILD,其特征是纤维化,主要发生在上肺部区域.
研究的目的:
- 在患有性结肠炎和复发性多重体炎的患者中报告一种罕见的多重体性纤维生化 (PPFE) 病例.
- 探索PPFE和性结肠炎之间的潜在关联.
- 强调在IBD患者中识别非典型肺部表现的重要性.
主要方法:
- 一个50多岁的女性的病例报告,有复发性多重体炎和性结肠炎的病史.
- 临床表现包括渐进性呼吸不全和干咳.
- 诊断工作包括体检,高分辨率CT (HRCT),肺功能测试 (PFT) 和肺组织的组织病理分析.
- 用普得尼索隆治疗和自发性肺胸的管理.
主要成果:
- 患者表现出呼吸不全和咳的症状,身体发现了胸和裂.
- 在HRCT中发现了双边的顶端膜加厚和上叶纤维化.
- 在PFT测试中,肺部表现出一种限制性肺部模式.
- 组织病理学证实了PPFE.
- 对普得尼索隆的初始反应,随后是疾病进展和呼吸衰竭.
结论:
- 这一案例表明,多发性膜纤维延展症 (PPFE) 和性结肠炎之间存在潜在的关联.
- 在IBD患者中,应考虑非典型的肺表现,包括PPFE.
- 提高意识和多学科方法对于IBD患者罕见ILD的诊断和管理至关重要.
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