Recent Advances in Idiopathic Pulmonary Fibrosis Noth Imre , MD, FCCP, Martinez Fernando J. , MD, MS, FCCP  CHEST  Volume 132, Issue 2, Pages 637-650 (August 2007) DOI: 10.1378/chest.06-1927 Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 1 Interobserver agreement is demonstrated among three expert clinicians as they are provided with incremental data (ie, clinical, radiologic, and pathologic) and interaction with expert radiologists and pathologists. Individuals made their diagnostic decisions without conferring in steps 1 and 2, and individually after conferring in steps 3 to 5. In stage 5, all participants came to a consensus opinion. From Flaherty et al.13 CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 2 HRCT images from a patient with histologically confirmed UIP and a clinically compatible picture of IPF. Peripheral honeycomb change is illustrated by a red arrow. CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 3 Kaplan-Meier survival curves for patients grouped by combining HRCT scan and histopathologic features as follows: histopathologic pattern showing NSIP and HRCT scan interpreted as indeterminate or NSIP (n = 23; dotted line); histopathologic pattern showing UIP and HRCT scan interpreted as indeterminate or NSIP (n = 46; dashed line); and a histopathologic pattern showing UIP and a HRCT scan interpreted as UIP (n = 27; solid line) [p = 0.001]. + = last follow-up visit; ○ = death. From Flaherty et al.22 CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 4 Top, A: Kaplan-Meier survival curves for patients with concordant UIP (UIP in all samples taken) [n = 51; dashed line], discordant UIP (UIP in at least one sample and other histology in at least one patient) [n = 28; solid line], and NSIP (n = 30; dotted line), grouped by histologic classification (p < 0.0003). + = last follow-up visit; ○ = death. From Flaherty et al.32Bottom, B: Kaplan-Meier survival curves for patients with concordant UIP (n = 25), discordant UIP-NSIP (n = 8), and concordant NSIP-NSIP (n = 31), grouped by histologic patterns. Patients with concordant NSIP-NSIP had significantly better survival than patients with both discordant UIP-NSIP and concordant UIP (p = 0.02 and 0.04, respectively), but there was no significant difference between patients with concordant UIP-UIP and those with discordant UIP-NSIP (p = 0.48). From Monaghan et al.34 CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 5 Algorithm outlining an approach for evaluating a patient with suspected interstitial lung disease (ILD). Hx = history; PE = physical examination; PFT = pulmonary function tests; bronch = bronchoscopy; Dx = diagnosis; RBILD = respiratory bronchiolitis, interstitial lung disease; DIP = desquamative interstitial pneumonia; DAD = diffuse alveolar damage; OP = organizing pneumonia; LIP = lymphocytic interstitial pneumonia. From Martinez et al.91 CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 6 Top, A: longitudinal change in FVC in patients with mild-to-moderate IPF who died while being treated with placebo in a randomized, therapeutic trial. Modest changes are noted. Bottom, B: longitudinal change in breathlessness measured with the University of California San Diego questionnaire in patients with mild-to-moderate IPF who died while being treated with placebo in a randomized, therapeutic trial. Rapid changes are noted in a subgroup of patients. From Martinez et al.5 CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions

Figure 7 Top left, A, and top right, B: HRCT scan images from a patient with confirmed IPF who is in a stable state. Bottom left, C, and bottom right, D: HRCT scan images from the same patient 2 months later during a time of worsening breathlessness and cough. Note the increased ground-glass opacity (arrows). No alternative etiology was identified for the findings, and immunosuppressive therapy was intensified. CHEST 2007 132, 637-650DOI: (10.1378/chest.06-1927) Copyright © 2007 The American College of Chest Physicians Terms and Conditions