Amira Pharmaceuticals, Inc. announced today that AM152, the company's lead LPA1 antagonist, has been granted an orphan drug designation by the U.S. Food and Drug Administration for the treatment of idiopathic pulmonary fibrosis. Commonly referred to as IPF,  
idiopathic pulmonary fibrosisis scarring or thickening of the lungs without a known cause.   This fibrotic disease affects the lungs of patients and their ability to breathe. 

No one knows what causes pulmonary fibrosis or why some people get it but the condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found.     
Activation of the LPA1 receptor by LPA has been implicated in a number of disease processes, including tissue fibrosis,  and AM152 is a lysophosphatidic acid (LPA) receptor 1 antagonist.   LPA1 receptor antagonists have displayed efficacy in a wide range of preclinical fibrosis models, including lung, skin, eye, liver and kidney. 

The disease occurs most often in people between 50 and 70 years old and in some people the disease gets worse quickly (over months to a few years), while others have little worsening of the disease over time.

"This is an important development for Amira, and potentially patients suffering from IPF, as we continue to move this promising therapeutic candidate forward in development," said Bob Baltera, Chief Executive Officer. "We are currently completing our Phase 1 studies with AM152 and expect to begin a Phase 2 study by the end of 2011 or in early 2012. Currently there are no FDA-approved therapies for IPF, and we look forward to better understanding the potential therapeutic benefit of an LPA1 antagonist in this disease area."