Pharmacological management of connective tissue disease-associated pulmonary arterial hypertension

Expert Opin Pharmacother. 2023 Oct 23. doi: 10.1080/14656566.2023.2273395. Online ahead of print.ABSTRACTINTRODUCTION: Pulmonary arterial hypertension (PAH) is a severe, progressive pulmonary vasculopathy (Group 1 Pulmonary Hypertension (PH 1)) that complicates the course of many connective tissue diseases (CTD) and significantly impacts the quality and length of life of patients. Due to the various tissues that can be affected by CTD, detailed testing is required to differentiate true PAH from other types of pulmonary hypertension caused by CTD such as left heart disease (Group 2 PH), pulmonary parenchymal disease (Group 3 PH), and chronic thromboembolic pulmonary hypertension (Group 4 PH). CTD-associated PAH is most frequently seen in systemic sclerosis but can also be seen with systemic lupus erythematosus, mixed connective tissues disease, primary Sjogren's syndrome, and inflammatory myopathies.AREAS COVERED: This review discusses the epidemiology of CTD-associated PAH, outlines the complex approach to obtaining a diagnosis, and finishes with an in-depth discussion on the current treatment paradigm with a focus on the pharmacological options available to patients. Focus is placed on the challenges faced in the treatment of CTD-associated PAH, in particular the decreased efficacy and poorer tolerance of pharmacological therapies and includes a discussion on the future investigational treatments that may alter the landscape of therapy.EXPERT OPINION: Despite significant adv...
Source: Expert Opinion on Pharmacotherapy - Category: Drugs & Pharmacology Authors: Source Type: research