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Idiopathic PAH Vasodilator Response Reflects Blood Flow Problems

Patients with idiopathic pulmonary arterial hypertension (PAH) who responded to an acute vasodilator challenge — a clinical test that can predict how severe the disease will become — had fewer baseline blood flow abnormalities compared with non-responders. The study appeared in a research letter published in Annals…

First Results From a National Registry for Pulmonary Hypertension in Russia

Initial results are now available from a national registry that has been established in Russia to track individuals with pulmonary hypertension (PH), including chronic thromboembolic pulmonary hypertension (CTEPH). The research was published in Russian in the journal Terapevticheskii Arkhiv (translated to Therapeutic Archive). PH is a serious disease involving high blood…

Pulmonary Arterial Hypertension in the US: How Much Does it Cost?

Pulmonary arterial hypertension (PAH)  is a rare condition characterized by high blood pressure of the lung arteries. It costs a great deal in human life lost and life impairment, but how much money is it costing to treat? A recent study published in BMC Health Services Research attempted to answer…

Adiponectin May Prevent Skin Fibrosis in Systemic Sclerosis

An international collaboration of researchers from the United States and China reported that adiponectin (APN), a protein that regulates glucose levels and fatty acid breakdown in the body, may be a naturally-occurring anti-fibrotic agent for systemic sclerosis (SSc). They presented their study at the 2014 American College of Rheumatology Meeting…

PFF, FDA Share Information On Pulmonary Fibrosis Using Survey Data

The Pulmonary Fibrosis Foundation (PFF) and the U.S. Food And Drug Administration will discuss data from a survey requested for the Patient-Focused Drug Development Meeting For Idiopathic Pulmonary Fibrosis, to be held on the 26th of September on the FDA’s White Oak Campus. According to the information…

Pulmonary Fibrosis Awareness Month Is Already On The Move

The Coalition for Pulmonary Fibrosis (CPF), which has chosen September as National Pulmonary Fibrosis Month, recently announced this year’s activities for increasing advocacy and awareness for the disease. According to the organization, the initiative, now in its 12th year, intends to raise awareness of the disease and…

Discovery Channel To Air Documentary on Idiopathic Pulmonary Fibrosis

Every Breath Counts: Idiopathic Pulmonary Fibrosis, a documentary to mark Pulmonary Fibrosis Awareness Month, will air on the Discovery Channel on September 13th and 27th at 8:00 am ET/PT. Supported by Boehringer Ingelheim Pharmaceuticals, Inc. and created together with the Pulmonary Fibrosis Foundation and the Coalition for Pulmonary Fibrosis, the…

Treating Inflammation in PAH Using Antioxidant Inflammation Modulators

Pulmonary Arterial Hypertension (PAH) is a life-threatening disease in which endothelial dysfunction, vasoconstriction in small pulmonary arteries, dysregulated proliferation of vascular cells, and dysregulated inflammatory signaling leads to pulmonary arterial obstruction increasing pulmonary vascular resistance and resulting in right ventricular failure. Different types of treatments for PAH exist that can…

New Study Outlines Potential PH Therapy Targets

Pulmonary hypertension is a condition caused by increased pressure in the pulmonary arteries. In advanced cases, its symptoms (shortness of breath, tiredness, chest pain) worsen and may limit all physical activity. Now, researchers from University of California, Los Angeles have developed a successful treatment to rescue advanced forms of…


A Conversation With Rare Disease Advocates