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Forced Vital Capacity Found To Be Inadequate When Assessing Scleroderma

Forced vital capacity, or FVC, is defined as the amount of air that can be forcibly exhaled from the lungs after taking the deepest breath possible. Patients suffering from systemic sclerosis, or scleroderma (a chronic systemic autoimmune disease) often undergo this type of spirometry testing to assess their status of…

Systemic Sclerosis Patients At Higher Risk To Develop PAH

A team of over twenty researchers recently found that patients with systemic sclerosis are at high risk to develop pulmonary arterial hypertension (PAH). The study, published in the Seminars in Arthritis & Rheumatism, set out to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (Ssc),…

Experimental Therapy Targets Inflammation in Pulmonary Arterial Hypertension

Reata Pharmaceuticals, Inc. is a privately held company located in Irving, Texas aiming to translate innovative research into breakthrough medicines for difficult-to-treat diseases that have significant unmet needs. Their approach specifically involves developing a novel class of drugs with potent transcriptional activity, called antioxidant inflammation modulators (AIMs), which can…

PAH Drug Developer Bellerophon Therapeutics Names Jonathan Peacock Chairman and Chief Executive Officer

Bellerophon Therapeutics, a biotherapeutics company working on a therapeutic treatment for pulmonary arterial hypertension, announced yesterday that Jonathan Peacock has been appointed chairman and chief executive officer. A graduate in Economics from the University of St. Andrews in Scotland, Peacock, who will replace Daniel Tassé in the role of chairman and…

Actelion Launches Opsumit For Pulmonary Arterial Hypertension in England

Swiss biotech company Actelion Pharmaceuticals has launched Opsumit (macitentan) as a new treatment option for patients with pulmonary arterial hypertension (PAH) in England, where approximately 3,000 people receive disease-targeted drug therapy for the condition. This comes as significant news for Actelion, after the Institut National d’Excellence en Santé…

Supernus Receives $2 Million for United Therapeutics’ Launch of Orenitram

United Therapeutics Corporation paid Supernus Pharmaceuticals a $2 million milestone payment for the launch of United’s Orenitram Extended-Release Tablets for the treatment of pulmonary arterial hypertension (PAH) in the United States. A form of pulmonary hypertension, PAH is caused by the abnormal functioning of pulmonary artetires, which carry blood…

Beta Blockers Show Positive Results As PAH Therapy In Early Study

Last week, during the annual meeting of the American Thoracic Society 2014, Jasmijn van Campen, MD, a staff pulmonologist at VU University Medical Center in Amsterdam gave an oral presentation on her recent work on treating idiopathic pulmonary arterial hypertension with beta blockers. “Despite the contraindications, beta blockers are prescribed for…

Pulmonary Thromboendarterectomy Procedure Can Improve PH Patient’s Health, According To Study

Researchers from the University of California, San Diego, presented a new study at the 2014 American Thoracic Society International Conference revealing the effects of pulmonary thromboendarterectomy (PTE) in patients with chronic pulmonary thromboembolic disease, suggesting a positive effect in patients’ conditions after the procedure. Daniel Crouch, MD, pulmonary and critical fellow…


A Conversation With Rare Disease Advocates