Update in pulmonary fibrosis 2018

The continuing struggle in the field of interstitial lung diseases (ILDs) has long been how best to classify disease on the basis of etiology or shared pathogenetic mechanisms. In the era of antifibrotic therapeutics of proven efficacy, accurate diagnosis is more important than ever. The articles published in 2018 reflect this ongoing debate in the medical community. The year 2018 led to new recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF) with active efforts to do the same for chronic hypersensitivity pneumonitis (CHP). Over the past two decades, international agreement on diagnostic criteria of IPF allowed a deeper understanding of disease mechanisms to be reached and facilitated the achievement of outstanding therapeutic goals. However, continual reclassification should not restrain efforts aimed at the discovery of therapies that can be beneficial for patients with diverse forms of progressive fibrotic disease, who so desperately need interventions that improve both quality of life and lifespan. With an exciting array of new therapeutic targets emerging from basic laboratory investigations, the responsibility of balancing careful phenotyping while addressing compelling clinical needs via efficient trial design will rest on the respiratory community. Additional work highlights the need for pulmonologists to be advocates for the protection and support of their patients. Within the scope of this paper, we review the latest advances in diagnosis, management, and pathogenesis of IPF, which is still the focus of most of the published research in the field of ILD. We then revise the most relevant evidence published on non-IPF fibrotic lung disease, acknowledging that in the future more attention should be focused on finding efficacious treatments for those patients with progressive disease, regardless of specific etiologies and strict classification schemes.