Cause is not known. Here is some information that may be of interest:
"Idiopathic pulmonary fibrosis (IPF) is a disease characterized by progressive scarring, or fibrosis, of the lungs, which leads to their deterioration and destruction. The cause of IPF is unknown, and currently there is no FDA approved treatment. IPF occurs primarily in people 50 to 70 years of age. Based on the published scientific literature, median survival time from diagnosis is two to five years in patients with IPF, and most patients die from the complications associated with the disease. We believe that there are approximately 83,000 patients with IPF in the United States, approximately two-thirds of whom have mild to moderate disease severity.
InterMune has the latest stage compound currently in development for the treatment of IPF. Enrollment for CAPACITY, the Phase 3 program evaluating pirfenidone as a possible therapeutic candidate for the treatment of patients with IPF, was completed in May 2007. "
I own shares in InterMune (more for their potential in Hep C drug IMI-191 which although it will arrive on market at least two years after VX-950, now called “Telaprevir” may be better. I own Vertex also.)
Here is link to presentation of InterMune today (12June08):
http://phx.corporate-ir.net/phoenix.zhtml?c=100067&p=irol-EventDetails&EventId=1870319
The same drug ("pirfenidone") will be available in Japan perhaps in January of 2009 by firm Shionogi who has the Japanese, Korean & Taiwan rights.
Below are my notes related to IPF. (Intermune likes to point out to investors at these types of conferences that Pulmanary Arterial Hypertension, PAH has 6 drugs on the market selling ~1 billion dollars annually, even though only has 20% of the incidence of IPF, which has no drugs available.):
Pirfenidone (oral for IPF which has no drug vs 6 for PAH’s >$1e9 sales & 5x PAH’s incidence. May target other fibrosis targets also)
Shionogi has Japanese, Korean & Taiwan rights. Filed in Japan in March07. (RoW owned by ITMN)* 18March Shionogi** reported:
• Good Clinical Practice inspections passed at both the clinical sites and at the company.
• Shionogi presented P2&3on 368 cases total at 52 wk “positive effect”# (p= ~0.035) at Am. Thoracic Soc. 20May08 and:
• Abstract available at
http://www.thoracic.org (Session C95, Publication Page A768).
• Shionogi expects a pirfenidone NDA approval in Japan and launch in their FY2008, (April 2008 til March 2009).
• Top-line results of 72 wk P3 CAPACITY in IPF in January of 2009.
• InterMune to make long term safety study with patients who have completed one of CAPACITY1or2, P3s which have 779 patients total. (95% detect prob. If 50% better; 85% detect p. if 40% better) Forced Vital Capacity is end point (but 2nd is P.Free.Survial)
Hoping for both US & Eu approval in late 2009 or early 2010.
Shionogi's results became publicly available two weeks ago (but InterMune has shared data with them for months). Here is link to InterMune review of the Japanese results:
http://phx.corporate-ir.net/phoenix.zhtml?c=100067&p=irol-EventDetails&EventId=1856807
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# “positive effect” - Unfortunately, Pirfenidone is far from a cure. It only decrease the speed with which IPF disease kills, and by much less that factor of two - see slide 12 at the first link above. IPF is a grim picture.