Pulmonary Hypertension Phenotypes in Systemic Sclerosis: The Right Diagnosis for the Right Treatment

Abstract

Systemic sclerosis is an auto-immune disease characterized by skin involvement that often affects multiple organ systems. Pulmonary hypertension is a common finding that can significantly impact prognosis. Molecular pathophysiological mechanisms underlying pulmonary hypertension in systemic sclerosis can be extremely heterogeneous, leading to distinct clinical phenotypes. In addition, different causes of pulmonary hypertension may overlap within the same patient. Since pulmonary hypertension treatment is very different for each phenotype, it is fundamental to perform an adequate diagnostic work-up to properly and promptly identify the prevalent mechanism underlying pulmonary hypertension in order to start the right therapies. When pulmonary hypertension is caused by a primary vasculopathy of the small pulmonary arteries, treatment with pulmonary vasodilators, often in an initial double-combination regimen, is indicated, aimed at reducing the mortality risk profile. In this review, we describe the different clinical phenotypes of pulmonary hypertension in the scleroderma population and discuss the utility of clinical tools to identify the presence of pulmonary vascular disease. Furthermore, we focus on systemic sclerosis-associated pulmonary arterial hypertension, highlighting the advances in the knowledge of right ventricular dysfunction in this setting and the latest updates in terms of treatment with pulmonary vasodilator drugs.

Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

“…Rarely, patients with SSc may develop chronic thromboembolic pulmonary hypertension (CTEPH) and be classified as WHO Group 4 PH. Distinguishing among the different mechanisms of SSc associated PH is important as treatment and prognosis are impacted by a timely and accurate diagnosis ( 14 , 19 , 20 ).…”

“…In recent years, there has been a greater focus on understanding the pathophysiologic mechanisms driving RV dysfunction and PH associated with SSc patients, as well as the poor outcomes linked to the presence of PH in this patient population. Essential to this focus is the thorough investigation of the etiology of PH in patients with SSc, which often includes diagnostic tests such as full pulmonary function testing, including diffusion capacity for carbon monoxide (DLCO) for PH-ILD, ventilation perfusion scanning (V/Q scan) for CTEPH, and computed tomography scanning for radiographic correlates of PVOD/PCH ( 19 ). Cardiac imaging is frequently used for this purpose as well as diagnosing and monitoring disease status.…”

Apoptosis Disorder, a Key Pathogenesis of HCMV-Related Diseases

“…Systemic sclerosis (SSc) is mainly characterized by skin involvement that often affects multiple organ systems [ 61 ]. Among the numerous pathogenetic factors, HCMV is the key factor enhancing the progress of SSc [ 62 ].…”

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

“…Systemic sclerosis (SSc) is a connective tissue disease characterized by immune and endothelial dysfunction, inflammation and fibrosis [1][2][3][4][5][6][7][8]. The main cause of death in SSc patients is the effect that collagen overproduction has on the internal organs, such as the pulmonary system [9][10][11][12].…”