E-Mail karger@karger.com Thematic Review Series 2013 Respiration 2013;86:1–4 DOI: 10.1159/000353571 Practical Issues and Challenges in Idiopathic Pulmonary Fibrosis Vincent Cottin a Philippe Camus b a Hospices Civils de Lyon, Hôpital Louis Pradel, Service de Pneumologie – Centre de référence national des maladies pulmonaires rares, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, et b Centre Hospitalier Universitaire de Dijon et Université de Bourgogne, Dijon, France made in recent years and established evidence, suggesting elements of discussion in areas where evidence is still lacking and reviewing challenges that remain. In this is- sue, Poletti et al. [13] review the current diagnostic ap- proach of IPF and further discuss the heterogeneity of the disease that encompasses various clinical presentations, complications, comorbidities and phenotypes, especially regarding familial pulmonary fibrosis, rapidly progress- ing disease and subclinical ‘early’ IPF, with each of them raising specific challenges for clinicians. They further critically review elements on which to discuss the indica- tion for lung biopsy, the potential future role for cryobi- opsies (a novel endoscopic technique that is currently un- der evaluation for diagnosing the usual interstitial pneu- monia pattern), evaluation of individual prognosis in clinical practice, and the controversial issue of possible IPF and its practical management. In the next article of the series, Antoniou and Wells [in preparation] will review the current knowledge on acute exacerbations of IPF, a dreadful complication, which warrants active biologic and clinical research [14– 19]. Hypotheses for etiology, a proposed approach for appropriate diagnosis and suggested management will be reviewed, as well as recently identified potential triggers such as viral infection, surgery, microaspiration, expo- Idiopathic pulmonary fibrosis (IPF) is a devastating disease, with a median survival from diagnosis of only 3 years [1]. Although it is a rare and somewhat orphan dis- ease [2], its incidence, prevalence and specific mortality are rising [3–6], further increasing the many challenges faced by both patients and chest physicians. Recent evi- dence-based recommendations have been produced jointly by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Latin American Thoracic Association, establishing standards for the diagnosis and management of IPF. Standardization of diagnosis criteria originally published in 2000 [7] and updated in 2011 [8] has set the stage for drug development, and one first drug (pirfenidone) has been approved in a number of countries, representing a significant achievement in the fight against IPF. Howev- er, IPF remains a diagnostic and therapeutic challenge [9–11], with often more questions raised than answers provided. Clinicians also face a number of practical situ- ations and decisions that are not fully addressed by inter- national guidelines [9, 12]. This issue of Respiration includes the first article of a thematic review series on Practical issues and challenges in IPF (table 1). This timely series authored by IPF experts will address selected practical issues, reviewing progress Published online: June 26, 2013 Vincent Cottin Hospices Civils de Lyon, Hôpital Louis Pradel Service de Pneumologie – Centre de référence national des maladies pulmonaires rares Université Claude Bernard Lyon 1, FR–69677 Lyon (France) E-Mail vincent.cottin  @  chu-lyon.fr © 2013 S. Karger AG, Basel 0025–7931/13/0861–0001$38.00/0 www.karger.com/res