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Original Research: Diffuse Lung Disease |

High-Resolution CT Scan Findings in Familial Interstitial Pneumonia Do Not Conform to Those of Idiopathic Interstitial PneumoniaCT Scan Finding in Familial Interstitial Pneumonia

Ho Yun Lee, MD; Joon Beom Seo, MD, PhD; Mark P. Steele, MD, FCCP; Marvin I. Schwarz, MD, FCCP; Kevin K. Brown, MD, FCCP; James E. Loyd, MD; Janet L. Talbert, MS; David A. Schwartz, MD; David A. Lynch, MD
Author and Funding Information

From the Division of Radiology (Drs Lee, Seo, and Lynch) and the Department of Medicine (Drs Schwarz, Brown, and Schwartz and Ms Talbert), National Jewish Health and University of Colorado, Denver, CO; the Department of Medicine (Dr Steele), Duke University Medical Center, Durham, NC; and the Division of Pulmonary (Dr Loyd), Vanderbilt University School of Medicine, Nashville, TN.

Correspondence to: David A. Lynch, MD, Division of Radiology, National Jewish Health and Research Center, 1400 Jackson St, Denver, CO 80206; e-mail: lynchd@njhealth.org


Dr Lee is currently at the Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.

Dr Seo is currently at the Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.

Funding/Support: This study was supported by grants from the National Heart, Lung and Blood Institute [RO1-HL095393 and RO1-HL097163], both to Dr Schwartz.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(6):1577-1583. doi:10.1378/chest.11-2812
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Background:  The aim of this study was to describe the high-resolution CT (HRCT) scan features that characterize familial interstitial pneumonia (FIP).

Methods:  FIP was defined by the presence of two or more cases of probable or definite idiopathic interstitial pneumonia (IIP) in individuals related within three degrees. The cases were collected consecutively from three centers. We identified 371 individuals with potential FIP from 289 families, including 340 individuals who had HRCT scans. Two chest radiologists independently reviewed the HRCT scans, scoring the extent and distribution of HRCT scan findings, and assessed the overall radiologic diagnosis.

Results:  HRCT scan abnormalities suggestive of IIP were present in 85% (289 of 340 subjects). The most frequent findings were reticular pattern (n = 238, 82%) and ground-glass opacity (GGO) associated with reticular abnormality (n = 231, 80%). Other changes included GGO in 116 (40%), honeycombing in 92 (32%), and micronodules in 65 (22%). In the 289 cases with evidence of IIP, the findings were diffusely distributed in the craniocaudal plane in 186 (64%), and the lower lung zones were predominantly involved in 89 (31%). In the axial plane, 194 (67%) had a subpleural distribution; 88 (30%) were diffuse. The imaging pattern was classified as definite or probable usual interstitial pneumonia (UIP) in only 62 subjects (22%) and definite or probable nonspecific interstitial pneumonia (NSIP) in 35 subjects (12%). In 160 subjects (55%), the imaging findings did not conform to previously described UIP or NSIP patterns.

Conclusions:  Reticulation and a mixed GGO/reticular pattern are the most common HRCT scan findings in FIP. The parenchymal abnormalities are most often diffuse in the craniocaudal dimension and have a predominantly peripheral distribution in the axial dimension. Although a radiologic UIP pattern is not uncommon, most cases do not conform to typical UIP or NSIP patterns.

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