title: The diagnosis of idiopathic pulmonary fibrosis: current and future approaches.
abstract: With the recent development of two effective treatments for patients with idiopathic pulmonary fibrosis, an accurate diagnosis is crucial. The traditional approach to diagnosis emphasises the importance of thorough clinical and laboratory evaluations to exclude secondary causes of disease. High-resolution CT is a critical initial diagnostic test and acts as a tool to identify patients who should undergo surgical lung biopsy to secure a definitive histological diagnosis of usual interstitial pneumonia pattern. This diagnostic approach faces several challenges. Many patients with suspected idiopathic pulmonary fibrosis present with atypical high-resolution CT characteristics but are unfit for surgical lung biopsy, therefore preventing a confident diagnosis. The state of the art suggests an iterative, multidisciplinary process that incorporates available clinical, laboratory, imaging, and histological features. Recent research has explored genomic techniques to molecularly phenotype patients with interstitial lung disease. In the future, clinicians will probably use blood-specific or lung-specific molecular markers in combination with other clinical, physiological, and imaging features to enhance diagnostic efforts, refine prognostic recommendations, and influence the initial or subsequent treatment options. There is an urgent and increasing need for well designed, large, prospective studies measuring the effect of different diagnostic approaches. Ultimately, this will help to inform the development of guidelines and tailor clinical practice for the benefit of patients.
authors.affiliation: Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medical College, New York-Presbyterian Hospital/Weill Cornell Medical Center, New York, NY, USA. Electronic address: fjm2003@med.cornell.edu.
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.affiliation: Respiratory Effectiveness Group, Cambridge, UK.
authors.forename: Alison
authors.initials: A
authors.lastname: Chisholm
authors.affiliation: Department of Medicine, University of California San Francisco, San Francisco, CA, USA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Department of Medicine, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Kevin R
authors.initials: KR
authors.lastname: Flaherty
authors.affiliation: Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Myers
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Department of Medicine, University of Washington, Seattle, WA, USA.
authors.forename: Ganesh
authors.initials: G
authors.lastname: Raghu
authors.affiliation: Department of Radiology, Royal Brompton Hospital, London, UK.
authors.forename: Simon L F
authors.initials: SL
authors.lastname: Walsh
authors.affiliation: Division of Pulmonary & Critical Care Medicine, Department of Medicine, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Eric S
authors.initials: ES
authors.lastname: White
authors.affiliation: Catholic University of the Sacred Heart, A. Gemelli University Hospital, Rome, Italy; Academic Unit of Clinical and Experimental Sciences, NIHR Southampton Respiratory Biomedical Research Unit, University Hospital Southampton, Southampton, UK.
authors.forename: Luca
authors.initials: L
authors.lastname: Richeldi
mesh_terms: D001706:Biopsy / Q000379:methods; D003937:Diagnosis, Differential; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis*; D008168:Lung / Q000473:pathology; D063189:Symptom Assessment / Q000379:methods*; D014057:Tomography, X-Ray Computed / Q000379:methods*