Eight Parameters Recommended to Monitor for PH in JSSc Patients

When young patients develop juvenile systemic sclerosis (JSSc), it is vital to monitor them for changes in cardiopulmonary parameters that may lead to conditions such as pulmonary hypertension. However, there are no current guidelines that describe which cardiopulmonary parameters are most vital to monitor in order to screen for diseases…

Researchers Discover Protein That Can Prevent Pulmonary Hypertension-Related Heart Failure

RegeneRx Biopharmaceuticals, Inc. announced a recent study where company’s scientists demonstrated that Thymosin beta 4 (TB4) can reduce or even prevent heart failure in a pulmonary hypertension mouse model. The study, entitled “Thymosin Beta 4 protects mice from monocrotaline-induced pulmonary hypertension and right ventricular hypertrophy,” was published…

SSc-PAH Patients Demonstrate Endothelial-to-Mesenchymal Transition

It is widely accepted that hypertrophic mesenchymal stem cells contribute to pulmonary arterial hypertension (PAH) in individuals with systemic sclerosis (SSc). Now, as a result of a study presented at the 2014 American College of Rheumatology Meeting, scientists have reason to believe that endothelial cells can increase the pool…

Boehringer Ingelheim’s Nintedanib for IPF Receives Positive Opinion

Boehringer Ingelheim, a leader in the pharmaceuticals industry, recently received a positive opinion from the Committee for Medicinal  Products for Human Use (CHMP) of the European Medicines Agency (EMA) for the use of nintedanib (suggested brand name OFEV®) in patients with idiopathic pulmonary fibrosis (IPF). Nintedanib is now on…

UC San Diego Health System Launches First US CTEPH Registry

The University of California San Diego Health System, a leader in chronic thromboembolic pulmonary hypertension (CTEPH) research and patient care, has just received a generous grant to fund the recent launch of the country’s first CTEPH registry. The $7.6 million grant comes from pharmaceutical giant Bayer HealthCare in time for the November Pulmonary…

Ikaria Signs New Partnership to Expand Inomax for Severe PH in Asia

Critical care company Ikaria, Inc. and biopharmaceutical company Lee’s Pharmaceutical Holdings Limited (Lee’s Pharm) have just signed signed a new agreement to drive the registration and commercial approval of INOMAX® (nitric oxide), a breakthrough inhaled treatment of hypoxic respiratory failure (HRF), in China, Hong Kong, Macau and Taiwan. The drug will be offered as a Total Care…

Novel Surgical CTEPH Treatment Approach Cures Disease

Vanderbilt Heart & Vascular Institute (VHVI) is offering a novel and specialized surgical approach for treating patients suffering from chronic thromboembolic pulmonary hypertension (CTEPH), a form of pulmonary hypertension (PH) that can cause breathing difficulties, acute pulmonary embolism, or blood clots in the lung. Even though the condition…

Statins Improve Efficacy, Safety Profile of Imatinib for PAH

Combining two drug therapies, statins and imatinib, may be more beneficial for pulmonary hypertension management than using either alone. The laboratory of Daria Orszulak-Michalak in Poland investigated co-administration of rosuvastatin or simvastatin with imatinib in rats with experimentally-induced pulmonary hypertension and found a benefit in reducing disease severity. Both…

Sildenafil Improves Pulmonary Hypertension Outcome in Premature Babies

November is Prematurity Awareness Month, and in light of World Prematurity Day celebrated yesterday, November 17, Nationwide Children’s Hospital shared with the pulmonary hypertension community the benefits of administering Sildenafil to premature newborn babies with pulmonary hypertension. Sildenafil is a phosphodiesterase-5 (PDE5) inhibitor more popularly…


A Conversation With Rare Disease Advocates