Is idiopathic pulmonary fibrosis - is environmental or genetic disease - an old wine in a new bottle of treatment

Abstract

Idiopathic Pulmonary Fibrosis (IPF) is a chronic inflammatory lung disorder which gradually process to establish fibrosis. This condition is found more commonly in men, but is rarest in younger than age 50 years and median age of diagnosis is about 65 years.1 Although the disease course is variable, unpredictable (some patients progress rapidly, other quite slowly and others have sudden worsening after periods of stability with nintedancib). The median survival time is 2-4 years after diagnosis. Though the incidence of this disease is low in India (0.5), and in the Asian countries (4.2) per 1 lack population per year, the incidence is very high in European and in north American countries, Canada etc and its incidence there is between 2.8-18 per 1 lack population per year. Anti-Inflammatory therapies with prednisolone, or prednisolone with immunosuppressive agents like azathioprine for long years did not improve the outcome rather was found putative and subsequent meta analysis with prednisolone or prednisolone with azathioprine or acytyl cysteine or warfarin or everolemus was later proved to be potentially harmful therapies. Treatment by bosenten, imatinib, mactraintan or sidenafil was also attempted since 2010-2015 but were considered potentially ineffective therapies for IPF. That are now accepted worldwide A tyrosine kinase inhibitors (rho-rho kinase pathway inhibitors) nintedanib and anti fibrogenic agent Pirfenidone (TGFB2 inhibitors). IPF is now generally regarded as a consequence of multiple interacting genetic and environmental risk factors with repetitive local micro injuries to aging alveolar epithelium. These micro injuries stimulate fibroblasts proliferation, produces extra cellular matrix expansions and altered matrix composition and biomechanics, induces matrix produces myofibroblast and aberrant remodeling of lung interstitium.2