Jonathan H Chung1, Christian W Cox2, Steven M Montner2, Ayodeji Adegunsoye3, Justin M Oldham3, Aliya N Husain4, Rekha Vij3, Imre Noth3, David A Lynch5, Mary E Strek3. 1. 1 Department of Radiology, The University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637. 2. 2 Department of Radiology, Mayo Clinic, Rochester, MN. 3. 3 Department of Medicine, Section of Pulmonary/Critical Care, The University of Chicago Medical Center, Chicago, IL. 4. 4 Department of Pathology, The University of Chicago Medical Center, Chicago, IL. 5. 5 National Jewish Health Main Campus, Denver, CO.
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.
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: Jonathan H Chung; Ayodeji Adegunsoye; Justin M Oldham; Rekha Vij; Aliya Husain; Steven M Montner; Ronald A Karwoski; Brian J Bartholmai; Mary E Strek Journal: Eur Radiol Date: 2021-04-13 Impact factor: 5.315
Authors: Darragh Brady; Eugene A Berkowitz; Amita Sharma; Jeanne B Ackman; Adam Bernheim; Michael Chung; Srihari Veeraraghavan; Brent P Little Journal: Radiol Cardiothorac Imaging Date: 2021-08-12
Authors: Marlee S Crews; Brian J Bartholmai; Ayodeji Adegunsoye; Justin M Oldham; Steven M Montner; Ronald A Karwoski; Aliya N Husain; Rekha Vij; Imre Noth; Mary E Strek; Jonathan H Chung Journal: J Clin Med Date: 2020-11-23 Impact factor: 4.241