Latin America Medical Department - GlaxoSmithKline, Estrada dos Bandeirantes, Rio de Janeiro, 8464, Brazil.
Pulmonary Hypertension Unit, Pulmonary Department - Heart Institute, University of Sao Paulo Medical School, Av. Dr. Eneas de Carvalho Aguiar, 44, Sao Paulo, 05403-000, Brazil.
BMC Pulm Med. 2018 Jun 26;18(1):106. doi: 10.1186/s12890-018-0667-8.
Pulmonary arterial hypertension is a rare, progressive disease with poor prognosis. However, there is limited information available on the characteristics of PAH patients outside of North America and Europe. This is particularly important as researchers have described that there are potential geographical and regional differences which are vital to consider in the design of clinical trials as well as PAH treatment. The aim of this study was to describe the epidemiology of PAH (PH group 1) in Latin America.
A search of electronic databases for studies published in English, Spanish or Portuguese was conducted specifying publication dates from the 1st of January 1987 until 10th October 2016. Two authors independently assessed papers for inclusion and extracted data. A narrative synthesis of the findings was conducted.
The search revealed 22 conference abstracts and articles, and on application of the inclusion criteria, six conference abstracts and articles were included in the final review. Studies/registries were based in Argentina, Brazil and Chile. In contrast to the available literature from developed countries, in Latin America, most patients were diagnosed at younger age; nevertheless, the higher prevalence of idiopathic PAH (IPAH) and the advanced stage of the disease at diagnosis were comparable to the existing literature, as the long term survival, despite the lower availability of targeted therapies.
This study highlights the regional characteristics in the epidemiology of group 1 PH. The recognition of these differences should be considered when developing clinical guidelines and extrapolating diagnostic and treatment algorithms. Equitable access to health care and therapies are also issues that need to be addressed in Latin America. Information coming from a large prospective registry representing the different populations in Latin America is of critical importance to increase disease awareness in the region and improve diagnosis and management.
肺动脉高压（PAH）是一种罕见的、进行性疾病，预后较差。然而，北美的信息对于研究欧洲以外的 PAH 患者的特征是有限的。这一点尤为重要，因为研究人员已经描述了存在潜在的地理和区域差异，在临床试验的设计以及 PAH 治疗中都需要考虑到这些差异。本研究旨在描述拉丁美洲 PAH（PH 组 1）的流行病学特征。
检索了 1987 年 1 月 1 日至 2016 年 10 月 10 日期间发表的英文、西班牙文或葡萄牙文的电子数据库，指定了出版物日期。两位作者独立评估论文的纳入标准并提取数据。对研究结果进行了叙述性综合。
检索结果共显示 22 篇会议摘要和文章，在应用纳入标准后，最终综述纳入了 6 篇会议摘要和文章。研究/注册中心位于阿根廷、巴西和智利。与发达国家现有的文献相比，在拉丁美洲，大多数患者的诊断年龄较小；然而，特发性 PAH（IPAH）的高发病率和诊断时疾病的晚期阶段与现有文献相似，尽管靶向治疗的可用性较低，但长期生存率仍然较高。
本研究强调了 PH 组 1 的流行病学的区域性特征。在制定临床指南和推断诊断和治疗算法时，应考虑到这些差异。在拉丁美洲，还需要考虑获得医疗保健和治疗的公平性。来自代表拉丁美洲不同人群的大型前瞻性登记处的信息对于提高该地区对疾病的认识以及改善诊断和管理至关重要。
