A medication already approved as a therapy for people with pulmonary hypertension (PH) is about to enter a second round of clinical testing to determine if it's safe for those with PH related to another lung disease known as idiopathic pulmonary fibrosis, or IPF.
Expanded Role Possible
CoTherix, the maker of Ventavis (iloprost) Inhalation Solution, announced in early June that it has initiated a Phase 2 clinical trial to test the drug in a group of patients with the two illnesses. "Ventavis is an ideal candidate to investigate for IPF patients with pulmonary hypertension," said James Pennington, MD, Executive Vice-President and Chief Medical Officer of CoTherix. "Since Ventavis is inhaled, we believe it targets only the non-fibrotic portions of the diseased lung, increasing [blood vessel dilation] only in the ventilated areas, and reducing the potential for mismatched ventilation and pulmonary bloodflow, which can lead to heart strain and fainting."
The primary objective of the double-blind, placebo-controlled clinical trial, called ACTIVE, will be to determine the safety of Ventavis in patients with PH who also have been diagnosed with mild to moderate idiopathic pulmonary fibrosis. Included in the study will be the standard 6-minute walk test, which determines how far a particular patient can walk in a given time period; and any changes in disease progression or blood circulation.
The study will involve about 50 patients at 15 sites in the United States, CoTherix stated.
Progressive Scarring Disease
Idiopathic pulmonary fibrosis is a disease characterized by progressive scarring of the lungs that gradually complicates breathing. When it reaches that point, the disease prevents organs from obtaining levels of oxygen that keep them functioning. IPF is one of 200 diseases that fall into a class known as interstitial lung disease (ILD), in which lung scarring is a key manifestation.
While IPF can be linked in some cases to certain causes like exposure to inorganic or organic dusts, chemotherapy or radiation therapy, lung infection, or certain connective tissue diseases, in most cases, it has no known cause.
It's estimated that approximately 83,000 people in the United States have IPF, and an estimated 31,000 new cases develop each year. The disease typically strikes between the ages of 50 and 70, and tends to affect men more than women. The key symptoms are shortness of breath and a dry cough.1 According to CoTherix, the average odds of survival for patients with the disease is approximately 4 to 6 years after diagnosis.
Pulmonary hypertension, in which the blood pressure in the pulmonary artery leading from the heart to the lungs rises to abnormal levels, can be a serious complication of IPF.2 It's estimated that about one-fifth of IPF patients also have been diagnosed with pulmonary hypertension.
'Limited' Therapy Choices
"Treatment options for these patients are extremely limited," said Richard Channick, MD, an associate professor of Medicine at the University of California, San Diego, an expert on pulmonary hypertension and its treatment who will be taking part in the upcoming clinical trial. "There is a significant need for new therapies to treat the pulmonary hypertension complications of this progressive disease. I am very excited about the potential for Ventavis in IPF, and look forward to participating in the study."
If approved, Ventavis would become the first available medication to treat PH associated with IPF. It was initially approved as a PH medication late last year.
1. Coalition for Pulmonary Fibrosis. What is IPF? Some Basic Facts. Available at: http://www.coalitionforpf.org/Patients/whatisipf.asp. Accessed July 7, 2005.
2. Olschewski H, Ghofrani HA, Walmrath D et al. Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis. Am J Respir Crit Care Med 1999 Aug;160(2):600-7.
John Martin is a long-time health journalist and an editor for Priority Healthcare. His credits include overseeing health news coverage for the website of Fox Television's The Health Network, and articles for the New York Post and other consumer and trade publications.
