Pulmonary arterial hypertension in systemic sclerosis: Diagnosis and treatment according to the European Society of Cardiology and European Respiratory Society 2015 guidelines

Abstract

Scleroderma (systemic sclerosis) is an autoimmune connective tissue disease which presents endothelial dysfunction and fibroblast dysregulation, resulting in vascular and fibrotic disorders. Pulmonary hypertension is frequent in patients with systemic sclerosis: the natural evolution of the disease can induce the development of different forms of pulmonary hypertension, representing one of the main causes of death. Among the different forms of pulmonary hypertension in systemic sclerosis, pulmonary arterial hypertension is the most frequent one (rate of occurrence is estimated between 7% and 12%). This pulmonary vascular complication should be treated with a combination of drugs that is able to counteract endothelial dysfunction, antagonizing the endothelin-1 system and replacing prostaglandin I 2 and nitric oxide activity. A correct diagnosis is mandatory, because it is possible only for pulmonary arterial hypertension to use specific drugs that are able to control the symptomatic condition and the evolution of the disease. According to the most recent guidelines, for the patients with systemic sclerosis, also without pulmonary hypertension symptoms, echocardiography screening for the detection of pulmonary hypertension is recommended. Pulmonary arterial hypertension screening programs in systemic sclerosis patients is able to identify milder forms of the disease, allowing earlier management and better long-term outcome.

An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients

“…Many valuable authoritative reviews have been written on the management of PAH and guidelines developed by the ESC/ERS for the diagnosis and treatment of PAH [14,64]. Giordano et al [65], review the relevance and impact of the 2015 ESC/ERS guidelines to patients with SSc-PAH. In this review we will focus on the major drug classes, including combination therapy, relevant to SSc-vasculopathy in SSc.…”

Systemic Sclerosis: From Pathophysiology to Novel Therapeutic Approaches

“…Substantial progress has also been made in the management of SSc complications in recent years, which has led to increased survival and quality of life. This includes better control of complications in specific organs (such as interstitial lung disease (ILD) [ 8 ], pulmonary arterial hypertension (PAH) [ 9 ], scleroderma renal crisis, and Raynaud’s phenomenon), as well as standardized follow-up and early diagnosis of potential complications [ 10 ].…”

Cardiac Catheterization versus Echocardiography for Monitoring Pulmonary Pressure: A Prospective Study in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

“…While standard echocardiography is an important non-invasive screening procedure for these patients, PAH diagnosis is only established by right heart catheterization (RHC) [5,6]. Follow-up of CTD patients with PAH includes standard echocardiography and RHC every 6-12 months, or 3-6 months after changes in therapy, according to the 2015 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines [7].…”