Idiopathic pulmonary fibrosis (IPF) is the most prevalent of the idiopathic interstitial pneumonias and its incidence seems to be increasing [1–4]. IPF is generally considered to be unresponsive to “standard” therapies and carries a poor prognosis with most patients dying within at least 5 years following diagnosis [5–7]. An accurate diagnosis of IPF is essential for the optimal management of patients with IPF. The original IPF management guidelines, published in 2000 [8], have been superseded recently by the evidence-based international recommendations jointly sponsored by the European Respiratory Society (ERS), the American Thoracic Association (ATS), the Japanese Respiratory Society (JRS) and the Latin American Thoracic Association (ALAT) [9]. Nonetheless, despite this and other major accomplishments over the last decade, the diagnosis and the management of IPF remain a major medical challenge. Diagnostic uncertainties remain due, at least in part, to variability in the natural history of the disease and co-existing complicating comorbidities, interpretation and widespread application of the guideline recommendations, and analysis of …