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CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis.

Authors :
Chung JH
Cox CW
Montner SM
Adegunsoye A
Oldham JM
Husain AN
Vij R
Noth I
Lynch DA
Strek ME
Source :
AJR. American journal of roentgenology [AJR Am J Roentgenol] 2018 Feb; Vol. 210 (2), pp. 307-313. Date of Electronic Publication: 2017 Nov 15.
Publication Year :
2018

Abstract

Objective: A substantial proportion of cases of usual interstitial pneumonia (UIP) are due to connective tissue disease (CTD)-associated interstitial lung disease (ILD). The purpose of this study was to determine whether specific CT findings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fibrosis (IPF) and whether these signs are associated with survival.<br />Materials and Methods: Adults visiting an ILD clinic from 2006 to 2015 enrolled in a research registry with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern at high-resolution CT were included in the study. In these subjects with CT findings of UIP due to either IPF or CTD-ILD, three CT findings anecdotally associated with CTD-ILD were assessed for diagnostic accuracy: the "straight-edge" sign, the "exuberant honeycombing" sign, and the "anterior upper lobe" sign. Survival assessments were performed with univariate and multivariable techniques.<br />Results: The subjects included 63 patients who had CTD-ILD and 133 patients who had IPF with a UIP pattern at CT. All three CT signs were significantly more common in subjects with CTD-ILD than those with IPF (prevalence, 22.2-25.4% for CTD-ILD, 6.0-12.8% for IPF; p = 0.028 to < 0.001). The highest specificity (94.0%) and sensitivity (25.4%) were seen for the straight-edge sign. No CT sign was associated with survival in multivariable analysis.<br />Conclusion: Although UIP is usually associated with IPF, the index of suspicion for CTD-ILD should be raised in the care of patients with any of the three CT signs. A thorough workup for CTD-ILD should be pursued, including referral to the rheumatology department.

Details

Language :
English
ISSN :
1546-3141
Volume :
210
Issue :
2
Database :
MEDLINE
Journal :
AJR. American journal of roentgenology
Publication Type :
Academic Journal
Accession number :
29140119
Full Text :
https://doi.org/10.2214/AJR.17.18384