Diagnostic and prognostic markers and treatment of connective tissue disease-associated pulmonary arterial hypertension: current recommendations and recent advances

Introduction: Pulmonary arterial hypertension (PAH), also referred to as group 1 pulmonary hypertension, occurs either primarily or in association with other diseases such as connective tissue diseases (CTD). Of CTD, systemic sclerosis (SSc), systemic lupus erythematosus and mixed connective tissue disease are commonly accompanied with PAH. It is of note that SSc-PAH is associated with distinctive histopathology, an unfavorable outcome, and a blunted responsiveness to modern PAH therapies. Areas covered: The data in articles published until May 2020 in peer-reviewed journals, covered by PubMed databank, are discussed. The current review introduces recent advances over the past years which have moved our understanding of CTD-PAH forward and discusses what we are currently able to do and what will be necessary in the future to overcome the yet unsatisfactory situation in the management of CTD-PAH, particularly in that of SSc-PAH. Expert opinion: A multifaceted and integrated approach would be crucial to improve the outcome of patients with SSc-PAH. The authors also propose a possible algorithm to classify and treat SSc patients with suspicion of pulmonary vascular disease.