Guiot J, Cornia O, Louis R
Service de Pneumologie, CHU Liège, Belgique.
Service de Pharmacie clinique, CHU Liège, Belgique.
Rev Med Liege. 2020 May;75(5-6):344-349.
Pulmonary arterial hypertension (PAH) is a rare disease, characterized by a progressive increase in pulmonary arterial pressure. The therapeutic management of PAH patients has evolved significantly over the past decades following the appearance of new specific therapies, but also the performance of multiple clinical studies in an otherwise rare pathology. As a result, the care is very well codified and makes it possible to treat all patients at best. To date, we can cite four therapeutic families: endothelin receptor antagonists (ERA), drugs that interfere with the cyclic guanosine monophosphate (cGMP) pathway such as phosphodiesterase type 5 inhibitors (PDE5i) or the stimulator of soluble guanylate cyclase, prostacyclin analogues, and, finally, calcium antagonists. The therapeutic approach, formerly sequential, has proven to be insufficient in favor of an aggressive and rapidly progressive upfront therapeutic approach, making it possible to greatly improve the morbidity and mortality of patients. In this context, early management remains the most appropriate attitude and justifies recourse, from the first symptoms, to a competence center.
肺动脉高压(PAH)是一种罕见疾病,其特征是肺动脉压力进行性升高。在过去几十年中,随着新的特异性疗法的出现以及在这种罕见疾病中开展的多项临床研究,PAH患者的治疗管理有了显著进展。因此,治疗方案有了很好的规范,能够尽可能地为所有患者提供最佳治疗。迄今为止,我们可以列举出四类治疗药物:内皮素受体拮抗剂(ERA)、干扰环磷酸鸟苷(cGMP)途径的药物,如5型磷酸二酯酶抑制剂(PDE5i)或可溶性鸟苷酸环化酶刺激剂、前列环素类似物,以及钙拮抗剂。以前采用的序贯治疗方法已被证明是不够的,转而采用积极且迅速推进的初始治疗方法,这使得患者的发病率和死亡率得到了大幅改善。在这种情况下,早期管理仍然是最合适的做法,并且从出现最初症状起就求助于专业中心是合理的。