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CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis.
AJR Am J Roentgenol. 2018 Feb; 210(2):307-313.AA

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.

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.

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.

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.

Authors+Show Affiliations

1 Department of Radiology, The University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.2 Department of Radiology, Mayo Clinic, Rochester, MN.2 Department of Radiology, Mayo Clinic, Rochester, MN.3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL.3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL.4 Department of Pathology, The University of Chicago Medical Center, Chicago, IL.3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL.3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL.5 National Jewish Health Main Campus, Denver, CO.3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

29140119

Citation

Chung, Jonathan H., et al. "CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis." AJR. American Journal of Roentgenology, vol. 210, no. 2, 2018, pp. 307-313.
Chung JH, Cox CW, Montner SM, et al. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. AJR Am J Roentgenol. 2018;210(2):307-313.
Chung, J. H., Cox, C. W., Montner, S. M., Adegunsoye, A., Oldham, J. M., Husain, A. N., Vij, R., Noth, I., Lynch, D. A., & Strek, M. E. (2018). CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. AJR. American Journal of Roentgenology, 210(2), 307-313. https://doi.org/10.2214/AJR.17.18384
Chung JH, et al. CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. AJR Am J Roentgenol. 2018;210(2):307-313. PubMed PMID: 29140119.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis. AU - Chung,Jonathan H, AU - Cox,Christian W, AU - Montner,Steven M, AU - Adegunsoye,Ayodeji, AU - Oldham,Justin M, AU - Husain,Aliya N, AU - Vij,Rekha, AU - Noth,Imre, AU - Lynch,David A, AU - Strek,Mary E, Y1 - 2017/11/15/ PY - 2017/11/16/pubmed PY - 2018/11/7/medline PY - 2017/11/16/entrez KW - connective tissue disease KW - idiopathic pulmonary fibrosis KW - survival KW - usual interstitial pneumonia SP - 307 EP - 313 JF - AJR. American journal of roentgenology JO - AJR Am J Roentgenol VL - 210 IS - 2 N2 - 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. 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. 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. 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. SN - 1546-3141 UR - https://www.unboundmedicine.com/medline/citation/29140119/CT_Features_of_the_Usual_Interstitial_Pneumonia_Pattern:_Differentiating_Connective_Tissue_Disease_Associated_Interstitial_Lung_Disease_From_Idiopathic_Pulmonary_Fibrosis_ L2 - http://www.ajronline.org/doi/full/10.2214/AJR.17.18384 DB - PRIME DP - Unbound Medicine ER -