CT features of the usual interstitial pneumonia pattern: Differentiating connective tissue disease-associated interstitial lung disease from idiopathic pulmonary fibrosis

Jonathan H. Chung, Christian W. Cox, Steven M. Montner, Ayodeji Adegunsoye, Justin Oldham, Aliya N. Husain, Rekha Vij, Imre Noth, David A. Lynch, Mary E. Strek

Research output: Contribution to journalArticle

14 Citations (Scopus)

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 specifc CT fndings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fbrosis (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 fndings of UIP due to either IPF or CTD-ILD, three CT fndings 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 signifcantly 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 specifcity (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.

Original languageEnglish (US)
Pages (from-to)307-313
Number of pages7
JournalAmerican Journal of Roentgenology
Volume210
Issue number2
DOIs
StatePublished - Feb 1 2018
Externally publishedYes

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Idiopathic Pulmonary Fibrosis
Connective Tissue Diseases
Interstitial Lung Diseases
Lung
Survival
Rheumatology
Registries
Patient Care
Referral and Consultation

Keywords

  • Connective tissue disease
  • Idiopathic pulmonary fbrosis
  • Survival
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

Cite this

CT features of the usual interstitial pneumonia pattern : Differentiating connective tissue disease-associated interstitial lung disease from idiopathic pulmonary fibrosis. / Chung, Jonathan H.; Cox, Christian W.; Montner, Steven M.; Adegunsoye, Ayodeji; Oldham, Justin; Husain, Aliya N.; Vij, Rekha; Noth, Imre; Lynch, David A.; Strek, Mary E.

In: American Journal of Roentgenology, Vol. 210, No. 2, 01.02.2018, p. 307-313.

Research output: Contribution to journalArticle

Chung, Jonathan H. ; Cox, Christian W. ; Montner, Steven M. ; Adegunsoye, Ayodeji ; Oldham, Justin ; Husain, Aliya N. ; Vij, Rekha ; Noth, Imre ; Lynch, David A. ; Strek, Mary E. / CT features of the usual interstitial pneumonia pattern : Differentiating connective tissue disease-associated interstitial lung disease from idiopathic pulmonary fibrosis. In: American Journal of Roentgenology. 2018 ; Vol. 210, No. 2. pp. 307-313.
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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 specifc CT fndings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fbrosis (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 fndings of UIP due to either IPF or CTD-ILD, three CT fndings 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 signifcantly 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 specifcity (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.",
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T2 - 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

AU - Husain, Aliya N.

AU - Vij, Rekha

AU - Noth, Imre

AU - Lynch, David A.

AU - Strek, Mary E.

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KW - Idiopathic pulmonary fbrosis

KW - Survival

KW - Usual interstitial pneumonia

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