Abstract

The current review provides the reader with an overview of the diagnosis of idiopathic pulmonary fibrosis (IPF) but is merely dedicated to the changed paradigm associated with treatment strategies in IPF, a dreadful, chronic, and irreversibly progressive fibrotic lung disease. The IPF is a disease affecting mainly the elderly population (>50 years), is a moderately common disease (affecting 20–50 people per 100,000), is limited to the lungs, and has usual interstitial pneumoonia (UIP) as its principal radiological/ pathological pattern. The final diagnoosis is established in the majority of cases by a reasonable constellation of clinical and radiological signs, characteristic of definite UIP, and by exclusion of other conditions known to cause analogous lung fibrosis. Compared to the past era, a considerable bulk of new knowledge has accumulated on IPF during the last decade. Former suggestions for treatment consisting of a combination of prednisolone, azathioprine or cyclophosphamide, and N-acetylcysteine (so-called “triple therapy”), which has been based on expert opinions, few low-quality studies, and a pressure to treat, has recently appeared deleterious rather than effective. This judgment is authentic because the triple combination caused increased risks of death and hospitalizations, compared with placebo. Instead, an antifibrotic agent, pirfenidone, has been shown to be effective in inhibiting the fall in lung function and progression of IPF. Moreover, there are numerous ongoing trials with potential novel drug candidates for IPF like monoclonal antibodies against various cytokines, integrins, and growth factors, as well as growth factor receptor-associated tyrosine kinase inhibitors. Non-pharmacological therapy and treatment of acute exacerbations of IPF is also discussed.