Al-Qadi Mazen, LeVarge Barbara, Ford H James
Division of Pulmonary and Critical Care Medicine, Pulmonary Hypertension Program, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States.
Front Med (Lausanne). 2021 Mar 25;7:616720. doi: 10.3389/fmed.2020.616720. eCollection 2020.
Pulmonary hypertension (PH) is recognized to be associated with a number of comorbid conditions. Based on these associations, PH is classified into 5 groups, considering common pathophysiologic drivers of disease, histopathologic features, clinical manifestations and course, and response to PH therapy. However, in some of these associated conditions, these characteristics are less well-understood. These include, among others, conditions commonly encountered in clinical practice such as sarcoidosis, sickle cell disease, myeloproliferative disorders, and chronic kidney disease/end stage renal disease. PH in these contexts presents a significant challenge to clinicians with respect to disease management. The most recent updated clinical classification schemata from the 6th World Symposium on PH classifies such entities in Group 5, highlighting the often unclear and/or multifactorial nature of PH. An in-depth review of the state of the science of Group 5 PH with respect to epidemiology, pathogenesis, and management is provided. Where applicable, future directions with respect to research needed to enhance understanding of the clinical course of these entities is also discussed.
肺动脉高压（PH）被认为与多种合并症相关。基于这些关联，考虑到疾病的常见病理生理驱动因素、组织病理学特征、临床表现和病程以及对PH治疗的反应，PH被分为5组。然而，在其中一些相关病症中，这些特征的了解较少。其中包括临床实践中常见的病症，如结节病、镰状细胞病、骨髓增殖性疾病以及慢性肾脏病/终末期肾病。在这些情况下，PH给临床医生的疾病管理带来了重大挑战。第六届世界肺动脉高压研讨会最新更新的临床分类方案将这些病症归类为第5组，突出了PH通常不明确和/或多因素的性质。本文对第5组PH在流行病学、发病机制和管理方面的科学现状进行了深入综述。在适用的情况下，还讨论了为增强对这些病症临床病程的理解所需开展的研究的未来方向。
