Familial idiopathic pulmonary fibrosis: serial high-resolution computed tomography findings in 9 patients

Journal of Computer Assisted Tomography
Osamu NishiyamaNestor L Müller

Abstract

The purpose of this study was to assess the high-resolution computed tomography (CT) findings of familial idiopathic pulmonary fibrosis (IPF). High-resolution CT scans obtained in 9 consecutive patients with biopsy-proven familial IPF were reviewed. The presence, distribution, and extent of various CT findings were evaluated. All 9 patients had ground-glass opacities, intralobular reticular opacities, and irregular thickening of the interlobular septa; 7 (78%) had traction bronchiectasis, 7 (78%) had small foci of consolidation; and 3 (33%) had honeycombing. The abnormalities involved mainly the lower lung zones in 6 patients and the upper lung zones in 2 patients and had no zonal predominance in 1 patient. Follow-up CT showed increased extent of disease in 8 patients. The high-resolution CT findings of familial IPF resemble those of nonfamilial IPF. Familial IPF has a lower prevalence of honeycombing and a lower prevalence of predominant lower lung zone distribution than nonfamilial IPF, however.

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Citations

Jul 27, 2010·American Journal of Respiratory and Critical Care Medicine·Coline H M van MoorselJan C Grutters
Oct 31, 2012·Archives of Pathology & Laboratory Medicine·Kevin O LeslieDavid A Schwartz
Jun 2, 2006·Proceedings of the American Thoracic Society·William E Lawson, James E Loyd
Oct 8, 2005·The Journal of Heart and Lung Transplantation : the Official Publication of the International Society for Heart Transplantation·Lucas LawTrevor J Williams
Nov 24, 2005·AJR. American Journal of Roentgenology·Carolina Althoff SouzaAndrew Churg
Feb 4, 2016·PloS One·Sergio HarariGiancarlo Cesana
Jun 16, 2017·European Respiratory Review : an Official Journal of the European Respiratory Society·Brett M ElickerTravis S Henry
Oct 24, 2014·Journal of Thoracic Imaging·Akiko TeradaYoichiro Kobashi

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