Abstract

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive condition of unknown etiology. Both the tomographic patterns and the pathologic anatomy correspond to usual interstitial pneumonia (UIP). Unfortunately, this is an irreversible disease with a variable clinical course that involves different phenotypes. Some patients are stable for a long time, while others present frequent acute exacerbations, or suffer a rapid decline and die. Not long ago, most treatment options for IPF patients focused on inflammation and lung fibrosis, relying on antiinflammatories and immunosuppressants. However, results observed for steroid, azathioprine, and Nacetylcysteine therapies proved ineffective and were associated with increased mortality. During the last 3 years, our understanding of the underlying mechanisms of IPF pathogenesis has evolved remarkably. There is new evidence about the treatment of this disease. New trials have been published and are breaking new ground in the management of IPF patients. There are also numerous ongoing clinical trials studying potential targets related to IPF treatment.

Keywords: Idiopathic pulmonary fibrosis, clinical trials, treatment.

Graphical Abstract