Volume 155, Issue 4, Pages e91-e96 (April 2019) A Nonsmoker Man in His 40s With a Diagnosis of Genetic-Related Idiopathic Pulmonary Fibrosis (Surfactant-Protein C Gene Mutation)  Antonin Fattori, MD, Mickael Ohana, MD, PhD, Sandrine Hirschi, MD, Romain Kessler, MD, Nicola Santelmo, MD, Nadia Nathan, MD, PhD, Marie-Pierre Chenard, MD, Stephane Raymond, MD, Marie Legendre, PharmD, PhD, Fiorella Calabrese, MD  CHEST  Volume 155, Issue 4, Pages e91-e96 (April 2019) DOI: 10.1016/j.chest.2018.12.015 Copyright © 2019 American College of Chest Physicians Terms and Conditions

Figure 1 A, Anterior-posterior and (B) lateral chest radiograph showing diffuse bilateral increased interstitial marking and volume loss, with significant apical involvement, consistent with pulmonary fibrosis. CHEST 2019 155, e91-e96DOI: (10.1016/j.chest.2018.12.015) Copyright © 2019 American College of Chest Physicians Terms and Conditions

Figure 2 A, Axial and (B) sagittal multiplanar reconstruction of the high-resolution CT scan. Extensive interlobular and intralobular reticulations are seen, with traction bronchiectasis. Sagittal reconstruction demonstrates an absence of apical-basal gradient, with significant involvement of the middle and upper lung. Substantial ground-glass opacities are present in the anterior and basal segments. Honeycombing is absent. CHEST 2019 155, e91-e96DOI: (10.1016/j.chest.2018.12.015) Copyright © 2019 American College of Chest Physicians Terms and Conditions

Figure 3 A, Panoramic microscopic view shows a typical usual interstitial pneumonia pattern with alternate areas of fibrotic remodeling with sparse areas of normal parenchyma. Hematoxylin and eosin staining, original magnification: ×12.5. B, At higher magnification, patchy distribution of the interstitial fibrosis and fibroblastic foci are seen (arrows on focus). Hematoxylin and eosin staining, original magnification: ×40. C, Moderate fibrosis, inflammation, and fibroblastic foci easily seen also in peribronchial areas. Hematoxylin and eosin staining, original magnification, ×100. D, Panoramic view in less remodeled lung parenchyma shows bridging fibrosis connecting two close bronchioles between them (blue bracket) and bronchiole with pleura (black bracket). Hematoxylin and eosin staining, original magnification, ×12.5. E, An abortive granuloma (circle) with multiple giant cells. Hematoxylin and eosin staining, original magnification, ×100. F, Picture shows florid peribronchiolar metaplasia. Hematoxylin and eosin staining, original magnification, ×100. CHEST 2019 155, e91-e96DOI: (10.1016/j.chest.2018.12.015) Copyright © 2019 American College of Chest Physicians Terms and Conditions

Figure 4 Minimum intensity projection reconstructions (5 mm) demonstrating (A) a relative subpleural sparing with minimal reticulations in the most basal segments and (B) focal areas of subnormal lung parenchyma in-between extensive ground-glass opacities, resulting in mosaic attenuation. CHEST 2019 155, e91-e96DOI: (10.1016/j.chest.2018.12.015) Copyright © 2019 American College of Chest Physicians Terms and Conditions