ACR CONVERGENCE 2020—In patients with connective tissue diseases, such as systemic sclerosis (SSc), pulmonary hypertension (PH) remains one of the leading causes of morbidity and mortality. In her lecture on Friday, Nov. 6, Pulmonary Hypertension: An Update, Mardi Gomberg-Maitland, MD, MSc, professor of medicine and medical director of the Pulmonary Hypertension Program at The George…
A prognostic tool developed to predict survival in patients with various forms of pulmonary arterial hypertension (PAH) is fairly accurate in predicting survival outcomes for many patients with PAH related to systemic sclerosis (SSc-PAH), according to a new study. However, the prognostic accuracy is less reliable for SSc-PAH patients with the highest risk of death….
CHICAGO—In systemic lupus erythematosus (SLE), most types of severe pulmonary manifestations are relatively rare, but it’s crucial they be caught because of the potentially dire consequences, said Richard Silver, MD, professor in the Rheumatology Division at the Medical University of South Carolina, Charleston, at the 2018 ACR/ARHP Annual Meeting. Pulmonary Manifestations Parenchymal disease—seen in the…
CHICAGO—About 30 years ago, pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) began to outpace renal crisis as the main causes of death in scleroderma (SSc). But treating these lung complications has proved vexing for clinicians. There is no easy way to predict who will develop PAH. There is no telltale antibody and no…
Pulmonary arterial hypertension (PAH) in connective tissue disease (CTD) features significant morbidity and mortality. Standard therapies with endothelin receptor antagonists and phosphodiesterase-5 inhibitors have shown some clinical improvement in patients, but these clinical improvements have proved modest when compared with other types of PAH. As our case below suggests, rituximab may show promise as a…
CHICAGO—Scleroderma patients with pulmonary arterial hypertension (PAH) might benefit from more aggressive therapy, an expert in the field said in a session on lung involvement in rheumatic diseases at the American College of Rheumatology’s 2015 State-of-the-Art Clinical Symposium in May. Newer trials—the SERAPHIN trial on macitentan, GRIPHON on selexipag, and AMBITION on an ambrisentan/tadalafil combination—show…
Research identifies specific mortality risk predictors among SSc-APAH patients
Strategies to diagnose and manage dyspnea in the scleroderma patient