肺静脉封闭性疾病:临床综述
Himanshu Deshwal1, Sauradeep Sarkar1, Atreyee Basu2
1Division of Pulmonary, Sleep and Critical Care Medicine, Department of Medicine, West Virginia University School of Medicine, Morgantown, WV, USA.
肺静脉封闭性疾病 (PVOD) 是一种致命的疾病,影响肺血管. 诊断依赖于成像和临床特征,由于活检风险,肺移植是唯一的治疗方法.
科学领域:
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
- 遗传学 是一个遗传学.
背景情况:
- 肺血管病包括肺动脉小动脉,毛细血管和静脉的疾病.
- 肺静脉封闭性疾病 (PVOD) 是一种进展性,致命的疾病,被归类为肺动脉高血压 (PAH),具有毛细血管/静脉参与.
- 肺毛细血管血栓炎和肺毛细血管血栓炎 (PCH) 在临床上相似,具有共同的风险因素,通常被称为PVOD/PCH.
研究的目的:
- 审查PVOD的临床表现,诊断和管理.
- 突出区分PVOD与其他形式的PAH的挑战.
- 强调有限的治疗选择和肺移植的作用.
主要方法:
- 对PVOD和相关疾病的临床文献的审查.
- 对诊断标准的分析,包括放射和组织学发现.
- 讨论风险因素,遗传突变 (EIF2AK4) 和治疗结果.
主要成果:
- 根据症状和血液动力学,PVOD在临床上无法与其他PAH形式区分.
- 诊断往往需要放射性证据 (中腺瘤病变,地面玻璃不透明) 和临床症状 (缺氧,DLCO降低) 由于肺活检的高出血风险.
- 特征性组织学显示小肺静脉狭窄/封闭.
结论:
- 肺血管扩散剂治疗可以导致肺,限制PVOD的治疗选择.
- 肺移植仍然是PVOD的唯一治愈治疗方法.
- 早期和准确的诊断对于患者管理至关重要,尽管存在诊断方面的挑战.
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