结缔组织疾病中的肺高血压:每个CTD专家应该知道的 - 但害怕问!
S Sanges1, V Sobanski1, N Lamblin2
1Université de Lille, U1286, INFINITE, Institute for Translational Research in Inflammation, 59000 Lille, France; Inserm, 59000 Lille, France; CHU de Lille, Département de Médecine Interne et Immunologie Clinique, 59000 Lille, France; Centre National de Référence Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), 59000 Lille, France; Health Care Provider of the European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ReCONNET), 59000 Lille, France.
肺高血压 (PH) 通过多种途径复杂化结合组织疾病 (CTD). 诊断需要对右心脏进行导管,在CTD患者中,治疗因PH原因而异.
科学领域:
- 心脏病学 心脏病学
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
背景情况:
- 肺高血压 (PH) 是结合性组织疾病 (CTD) 的严重并发症,特别是系统性硬化症 (SSc),系统性红斑狼 (SLE) 和混合结合性组织疾病 (MCTD).
- 肺血管疾病中的PH源于多种多样和复杂的机制,包括肺血管病变 (PAH),左心脏病,慢性肺病和慢性血栓塞性PH.
- 早期怀疑PH的症状通常是由临床症状,心声回声检测结果和生物标志物表明的,但最终的诊断依赖于右心导管 (RHC).
研究的目的:
- 审查连接组织疾病患者肺高血压的机制,诊断和管理.
- 突出不同CTD亚型的PH的诊断挑战和治疗策略.
- 强调需要根据CTD患者PH的潜在原因进行量身定制的治疗.
主要方法:
- 关于结缔组织疾病中的肺高血压现有文献的综述.
- 对CTD中PH的诊断标准和查策略的分析.
- 对CTD群体内的不同PH群体的当前治疗方法的综合分析.
主要成果:
- 在CTD中PH可以通过各种机制表现出来 (组1-4PH),往往是复杂的联系.
- 虽然SSc-PAH的查策略已经确立,但其他CTD的数据有限.
- 在CTD中治疗PH是取决于机制的,从PAH特异性疗法到潜在疾病的管理和抗凝药.
结论:
- 肺高血压是CTD的一个重要问题,需要通过RHC进行准确的诊断.
- 在CTD中对PH的管理策略必须根据具体的底层机制进行个性化.
- 定期的患者随访对于优化所有CTD-PH患者的治疗结果至关重要.
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