Interstitial, Inflammatory, and Occupational Lung Disease/Obstructive Airways DiseaseThe Diagnosing and Staging of Idiopathic Pulmonary FibrosisGarthwaite, Helen S. MBBS, BSc, MRCP*; Maher, Toby M. MB MSc, PhD, MRCP*,†,‡Author Information *Interstitial Lung Disease Unit, Royal Brompton Hospital †National Heart and Lung Institute, Imperial College London ‡Center for Respiratory Research, University College London, Rayne Institute, London, UK T.M.M has received consultancy or advisory board fees from Sanofi-Aventis, Boehringer Ingelheim, GSK, Novartis, and Phillips Respironics. He has received travel reimbursement from Boehringer Ingelheim and speaker’s fees from Astra Zeneca and UCB. T.M.M.’s institution has received an unrestricted academic industry grant from GSK. H.S.G. declares that there is nothing to disclose. Address correspondence to: Toby M. Maher, MB MSc, PhD, MRCP, Interstitial Lung Disease Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. E-mail: email@example.com. Clinical Pulmonary Medicine: November 2012 - Volume 19 - Issue 6 - p 254-261 doi: 10.1097/CPM.0b013e318271c603 Buy Metrics Abstract Idiopathic pulmonary fibrosis (IPF) is the most frequently occurring interstitial lung disease. The condition tends to be inexorably progressive and conveys a poor prognosis with a median survival of 2.8 to 4.2 years. The dismal prognosis coupled with recent exciting clinical trial advances have made it more important than ever that IPF is correctly distinguished from other interstitial lung diseases at an early stage. Correct diagnosis of IPF relies on the careful integration of clinical evaluation, high-resolution computerized topography appearances, and in the minority of cases, surgical lung biopsy findings. The need to incorporate information from different sources has led to multidisciplinary team (MDT) assessment gaining primacy in the diagnosis of IPF. Clinical investigations in IPF, including lung function testing, 6-minute walk, and echocardiography, also enable an accurate assessment to be made concerning baseline disease severity and permit judgments to be made about short to medium-term prognosis. This article addresses the key issues encountered by physicians when assessing a patient with suspected IPF and highlights the value of key clinical investigations in the evaluation of patients with this devastating disease. © 2012 Lippincott Williams & Wilkins, Inc.