Challenges In Idiopathic Interstitial Lung Disease: An Update

Idiopathic interstitial pneumonias (IIPs) are a group of heterogeneous parenchymal lung disorders whose classification has been recently revised. Clinical reasoning and multidisciplinary approach are strongly recommended to achieve an optimal management of patients. Idiopathic pulmonary fibrosis (IPF), the most common entity among the IIPs, is associated with a poor prognosis and with many diagnostic and therapeutic challenges. From the pathogenesis to the prognosis, from the diagnosis to the treatment, many are the fields that still hide pitfalls in daily clinical practice, and the research is called to shed light on all of them to allow clinicians to walk on a safe ground. Growing research efforts aim at understanding, recognizing, treating and preventing this dreadful disease, thus providing answers to everyday clinical questions. Although these challenges look daunting when accounted all together, many promising steps forward have been achieved in recent years. Not only clinicians have not set limits to challenges, but had challenged and still are challenging these limits, hoping to overcome soon the greatest challenge: finding the cure for IPF. The aim of this review was to summarize recent progresses and latest findings about pathogenetic mechanisms, diagnostic options, treatment strategies and biomarker research in IPF and to provide an overview of recent advances on acute IPF exacerbations.