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Original Research: INTERSTITIAL LUNG DISEASE |

Pathologic and Radiologic Differences Between Idiopathic and Collagen Vascular Disease-Related Usual Interstitial Pneumonia

Jin Woo Song, MD; Kyung-Hyun Do, MD; Mi-Young Kim, MD; Se Jin Jang, MD; Thomas V. Colby, MD, FCCP; Dong Soon Kim, MD, FCCP
Author and Funding Information

From the Departments of Pulmonary and Critical Care Medicine (Drs. Song and D.S. Kim), Radiology (Drs. Do and M.-Y. Kim), and Pathology (Dr. Jang), Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea; and the Department of Pathology (Dr. Colby), Mayo Clinic, Rochester, MN.

Correspondence to: Dong Soon Kim, MD, FCCP, Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Pungnap-2dong, Songpa-gu, Seoul, South Korea; e-mail: dskim@amc.seoul.kr


The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(1):23-30. doi:10.1378/chest.08-2572
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Background:  Patients with usual interstitial pneumonia (UIP) associated with collagen vascular disease (CVD) have been reported to have a better prognosis than those with idiopathic pulmonary fibrosis with a UIP pattern (IPF/UIP) seen on histology. The aim of this study was to evaluate the pathologic and radiologic differences between the two conditions and their relationship with clinical outcome.

Methods:  A retrospective review of 100 patients (CVD-UIP, 39 patients; IPF/UIP, 61 patients) with UIP pattern diagnosed by surgical lung biopsy at one tertiary referral center.

Results:  The median follow-up period was 34.4 months. The CVD-UIP group was younger, included more women and nonsmokers, and showed better survival than the IPF/UIP group. Pathologically, CVD-UIP patients had fewer fibroblastic foci and smaller honeycombing (HC) spaces with higher germinal centers and total inflammation scores than IPF/UIP patients. Radiologically, CVD-UIP patients had a lower emphysema score and more likely a nontypical UIP pattern without HC. The germinal centers score was the best distinguishing feature between CVD-UIP and IPF/UIP patients (odds ratio, 2.948; p = 0.001) and was marginally related to survival (p = 0.076). The HC score (hazard ratio [HR], 1.134; p < 0.001), total lung capacity (TLC) [HR, 0.932; p = 0.004], and age (HR, 1.052; p = 0.017) were significant predictors of survival in all patients with UIP histology, regardless of the presence of CVD. Among IPF/UIP patients, those with positive autoantibodies were pathologically more similar to CVD-UIP than to IPF/UIP without autoantibodies, despite no difference in survival between them.

Conclusions:  The germinal centers score was the best discriminative between CVD-UIP and IPF/UIP patients; it was of marginal prognostic significance. Age, TLC, and HC score were independent prognostic factors in all patients with UIP histology.

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