title: CT Findings, Radiologic-Pathologic Correlation, and Imaging Predictors of Survival for Patients With Interstitial Pneumonia With Autoimmune Features.
abstract: OBJECTIVE
  The objective of this study is to determine the CT findings and patterns of interstitial pneumonia with autoimmune features (IPAF) and to assess whether imaging can predict survival for patients with IPAF.
  
  
  METHODS
  The study included 136 subjects who met the criteria for IPAF and had diagnostic-quality chest CT scans obtained from 2006 to 2015; a total of 74 of these subjects had pathologic samples available for review within 1 year of chest CT examination. CT findings and the presence of an usual interstitial pneumonitis (UIP) pattern of disease were assessed, as was the UIP pattern noted on pathologic analysis. Analysis of chest CT findings associated with survival was performed using standard univariate and multivariate Cox proportional hazards methods as well as the unadjusted log-rank test. Survival data were visually presented using the Kaplan-Meier survival curve estimator.
  
  
  RESULTS
  Most subjects with IPAF (57.4%; 78/136) had a high-confidence diagnosis of a UIP pattern on CT. Substantially fewer subjects (28.7%; 39/136) had a pattern that was inconsistent with UIP noted on CT. The presence of a UIP pattern on CT was associated with smoking (p < 0.01), male sex (p < 0.01), and older age (p < 0.001). Approximately one-fourth of the subjects had a nonspecific interstitial pneumonitis pattern on CT. Of interest, nearly one-tenth of the subjects had a CT pattern that was most consistent with hypersensitivity pneumonitis rather than the customary CT patterns ascribed to lung disease resulting from connective tissue disease. Most subjects with a possible UIP pattern on CT (83.3%) had UIP diagnosed on the basis of pathologic findings. Focused multivariate analysis showed that honeycombing on CT (hazard ratio, 2.17; 95% CI, 1.05-4.47) and pulmonary artery enlargement on CT (hazard ratio, 2.08; 95% CI, 1.02-4.20) were independent predictors of survival.
  
  
  CONCLUSIONS
  IPAF most often presents with a UIP pattern on CT and is associated with worse survival when concomitant honeycombing or pulmonary artery enlargement is present.
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Jonathan H
authors.initials: JH
authors.lastname: Chung
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Montner
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Ayodeji
authors.initials: A
authors.lastname: Adegunsoye
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Cathryn
authors.initials: C
authors.lastname: Lee
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Justin M
authors.initials: JM
authors.lastname: Oldham
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Aliya N
authors.initials: AN
authors.lastname: Husain
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Heber
authors.initials: H
authors.lastname: MacMahon
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Imre
authors.initials: I
authors.lastname: Noth
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Rekha
authors.initials: R
authors.lastname: Vij
authors.affiliation: 1 Department of Radiology, University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.|2 Section of Pulmonary/Critical Care, Department of Medicine, University of Chicago Medical Center, Chicago, IL.|3 Section of Pulmonary/Critical Care, Department of Medicine, University of California at Davis, Sacramento, CA.|4 Department of Pathology, University of Chicago Medical Center, Chicago, IL.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Strek
mesh_terms: D001327:Autoimmune Diseases / Q000000981:diagnostic imaging* / Q000401:mortality* / Q000473:pathology; D002641:Chicago / Q000453:epidemiology; D015897:Comorbidity; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000401:mortality* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D015995:Prevalence; D011379:Prognosis; D015203:Reproducibility of Results; D012189:Retrospective Studies; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D013223:Statistics as Topic; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed / Q000706:statistics & numerical data*
keywords: CT; connective tissue disease; interstitial pneumonia with autoimmune features; survival; usual interstitial pneumonitis