Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology, closely associated with interstitial lung disease that shows similar symptoms but with different mode of action. The disease is known so because it primarily involves the interstitium, where scars develop and progresses towards the center of the lungs, making breathing difficult for the patient. It develops primarily in older adults, and is sometimes associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia.
There is currently no drug especially for IPF, and no procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and breathlessness. Pirfenidone and Nintedanib have both been shown to slow the progression of IPF; however, some patients cannot take these medications due to their side effects.
On the basis of above mentioned circumstances, researchers are coming forward for the discovery of more and more drugs. Drug pipeline of this disease has 58 active drugs; however, it will take some time to see them launched into the market. Only one drug is available commercially, and that is Thrombomodulin Alfa (ART-123). It is a Phase III drug of Asahi Kasei Pharma Corporation, already marketed in Japan under the name of Recomodulin for the treatment of disseminated intravascular coagulation (DIC). 14 drugs are in Phase II; whereas the preclinical and discovery stage has approximately 30 drugs. It can be said that the road to a better treatment of IPF is a long one, with many drugs on the way to reach the stage of becoming a treatment option for IPF patients.