In the latest issue of The Lancet Respiratory Medicine, Ganesh Raghu and colleagues report the prevalence of idiopathic pulmonary fibrosis (IPF) in the United States in 2011 (494.5 cases/100,000 people, more than double the 2001 rate) in people aged 65 years and older. Its prevalence has increased each year, but the incidence remained stable between 2001 and 2011 (93.7/100,000 person-years), suggesting that people with IPF may be surviving longer. This conclusion is further supported by new data indicating that newly diagnosed patients have an average survival time of 3.8 years, an increase from the 2001 assessment level. Data obtained from other parts of the world are more moderate – in Europe, the prevalence of the disease is 23.4/100,000 person-years; in the UK the prevalence is only 7.44/100,000 person-years across all age groups. These figures aside, the continued increase in prevalence, longer patient survival times and an aging global population mean that IPF will become a very serious global disease burden. IPF has always been a challenge for respiratory physicians. Once diagnosed, patients face a poor prognosis, a paucity of effective treatments, and no cure. approved and was used in the clinical treatment of IPF. The drug was subsequently approved for use in Europe in 2011 and Canada in 2012. On May 18, Talmadge King et al. reported the results of a trial showing a decrease in disease progression rates, which they hoped would lead to FDA approval of pirfenidone in the United States. However, the drug did not reduce patients’ symptoms and improve their quality of life, and unpublished post-marketing-related data have increased concerns about patient adherence to the drug. Additional trials to test the long-term efficacy of the drug, such as survival and quality of life, are needed to improve patient adherence and acceptability. Encouragingly, a new and successful research study has emerged. Also on May 18, LucaRicheldi and colleagues tested the efficacy of nintedanib (a tyrosine kinase inhibitor) in 1,066 IPF patients, and the results were very promising: nintedanib suppressed the rate of decline in lung function and acute deterioration, and its side effects were tolerable (although patients reported a period during the 52-week trial when diarrhea occurred, but only 5% of the trial dropped out). In addition to these encouraging data, discoveries about the genomic biology of IPF are ongoing. In this issue of The Lancet Respiratory Medicine, MeiLan Han and her colleagues report the results of an analysis of lung microbes in IPF, identifying two microbial markers associated with Staphylococcus and Streptococcus spp. that are associated with disease progression. Also in this issue, Bridget Stuart and his colleagues reported that short telomere length was associated with lower survival in IPF patients. These early data point to potential therapeutic targets that may be able to increase future treatment options for IPF and improve understanding of the etiology of the disease. Along with the many positive developments in IPF, further questions arise. It is extremely important to properly consider the first questions that need to be analyzed next. We must identify the populations for which the drugs are indicated; pirfenidone and nintedanib have not been evaluated in populations other than patients with mild to moderate pulmonary dysfunction, and there is an urgent need to identify patients in the earliest stages of disease progression. Identifying good drug combinations to mitigate side effects will be key to the success of conducting long-term therapy, and maximizing treatment effects in patients with co-morbidities will be a key challenge. Other exciting findings include the identification of alternative diagnostic strategies for televised thoracoscopic surgery and coexistence studies for gastroesophageal reflux disease (GERD). As with other respiratory diseases, genetic technology will be an enabling tool for individualized management of patients with IPF. The perception of IPF has changed. In the next 5-10 years, there will be a revolutionary outlook on this poorly understood, deadly disease. Patients and physicians alike are waiting with bated breath to see what comes next.