Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune
connective-tissue disease. Once considered an untreatable and unpredictable condition …

The transcription factor NF-E2 p45-related factor 2 (NRF2; encoded by NFE2L2) and its
principal negative regulator, the E3 ligase adaptor Kelch-like ECH-associated protein 1 …

Pulmonary arterial hypertension leads to significant impairment in haemodynamics, right
heart function, exercise capacity, quality of life and survival. Current therapies have …

Pulmonary hypertension (PH) encompasses a syndrome of diseases that are characterized
by elevated pulmonary artery pressure and pulmonary vascular remodeling and that …

Systemic sclerosis (SSc) is a rare, systemic autoimmune disease of unknown etiology.
Among the systemic rheumatic diseases, SSc carries the highest mortality, in part due to the …

Abstract Sphingosine 1-phosphate (S1P), which acts via G protein-coupled S1P receptors
(S1PRs), is a bioactive lipid essential for vascular integrity and lymphocyte trafficking. The …

Registries have provided a wealth of information on the clinical and disease characteristics
of patients living with pulmonary arterial hypertension (PAH) since the 1980s. Certain PAH …

Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to
many cancers. Two randomized controlled trials of autologous hematopoietic stem cell …

BACKGROUND: Patients with pulmonary arterial hypertension (PAH) associated with
systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with …

Background Pulmonary arterial hypertension (PAH) is a leading cause of death in patients
with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes …