Interstitial pneumonia related to undifferentiated connective tissue disease: Pathologic pattern and prognosis

Ho Cheol Kim, Wonjun Ji, Mi Young Kim, Thomas V. Colby, Se Jin Jang, Chang Keun Lee, Seung Bong Han, Dong Soon Kim

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Background: Undifferentiated connective tissue disease (UCTD) involves conditions characterized by both having symptoms of connective tissue disease (CTD) and autoantibodies but not fulfilling the criteria of a specific CTD. The frequency or prognosis of the usual interstitial pneumonia (UIP) pattern in UCTD is unknown, which may be confused with idiopathic pulmonary fibrosis (IPF). This study aimed to investigate the frequency of the UIP pattern in interstitial pneumonia related to UCTD and compare its prognosis with that of IPF and UCTD-nonspecific interstitial pneumonia (UCTD-NSIP). Methods: The medical records of 788 patients presumptively diagnosed with idiopathic interstitial pneumonia at Asan Medical Center from January 2005 to December 2012 were retrospectively reviewed. UCTD was diagnosed according to the criteria by Corte and colleagues, and the prognoses were compared between UCTD-UIP and UCTD-NSIP and between UCTD-UIP and IPF. Results: Among 105 patients with UCTD (13.3% of total subjects), 44 had a UIP pattern (by surgical lung biopsy: 24; by high-resolution CT scan: 20), 29 had a nonspecific interstitial pneumonia pattern (by surgical lung biopsy), and nine had an organizing pneumonia pattern (by biopsy). The overall survival of the UCTD-UIP group was shorter than that of the UCTDNSIP group (P = .021) but significantly better than that of the IPF group (P = .042). Conclusions: A UIP pattern, which seems to be frequent in UCTD, showed a poorer prognosis than that of UCTD-NSIP. However, the prognosis of UCTD-UIP was significantly better than that of IPF, highlighting the importance of searching for underlying UCTD in suspected IPF cases.

Original languageEnglish (US)
Pages (from-to)165-172
Number of pages8
JournalChest
Volume147
Issue number1
DOIs
StatePublished - Jan 1 2015

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Connective Tissue Diseases
Interstitial Lung Diseases
Idiopathic Pulmonary Fibrosis
Biopsy
Idiopathic Interstitial Pneumonias
Lung

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine
  • Medicine(all)

Cite this

Kim, H. C., Ji, W., Kim, M. Y., Colby, T. V., Jang, S. J., Lee, C. K., ... Kim, D. S. (2015). Interstitial pneumonia related to undifferentiated connective tissue disease: Pathologic pattern and prognosis. Chest, 147(1), 165-172. https://doi.org/10.1378/chest.14-0272

Interstitial pneumonia related to undifferentiated connective tissue disease : Pathologic pattern and prognosis. / Kim, Ho Cheol; Ji, Wonjun; Kim, Mi Young; Colby, Thomas V.; Jang, Se Jin; Lee, Chang Keun; Han, Seung Bong; Kim, Dong Soon.

In: Chest, Vol. 147, No. 1, 01.01.2015, p. 165-172.

Research output: Contribution to journalArticle

Kim, HC, Ji, W, Kim, MY, Colby, TV, Jang, SJ, Lee, CK, Han, SB & Kim, DS 2015, 'Interstitial pneumonia related to undifferentiated connective tissue disease: Pathologic pattern and prognosis', Chest, vol. 147, no. 1, pp. 165-172. https://doi.org/10.1378/chest.14-0272
Kim, Ho Cheol ; Ji, Wonjun ; Kim, Mi Young ; Colby, Thomas V. ; Jang, Se Jin ; Lee, Chang Keun ; Han, Seung Bong ; Kim, Dong Soon. / Interstitial pneumonia related to undifferentiated connective tissue disease : Pathologic pattern and prognosis. In: Chest. 2015 ; Vol. 147, No. 1. pp. 165-172.
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AU - Colby, Thomas V.

AU - Jang, Se Jin

AU - Lee, Chang Keun

AU - Han, Seung Bong

AU - Kim, Dong Soon

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AB - Background: Undifferentiated connective tissue disease (UCTD) involves conditions characterized by both having symptoms of connective tissue disease (CTD) and autoantibodies but not fulfilling the criteria of a specific CTD. The frequency or prognosis of the usual interstitial pneumonia (UIP) pattern in UCTD is unknown, which may be confused with idiopathic pulmonary fibrosis (IPF). This study aimed to investigate the frequency of the UIP pattern in interstitial pneumonia related to UCTD and compare its prognosis with that of IPF and UCTD-nonspecific interstitial pneumonia (UCTD-NSIP). Methods: The medical records of 788 patients presumptively diagnosed with idiopathic interstitial pneumonia at Asan Medical Center from January 2005 to December 2012 were retrospectively reviewed. UCTD was diagnosed according to the criteria by Corte and colleagues, and the prognoses were compared between UCTD-UIP and UCTD-NSIP and between UCTD-UIP and IPF. Results: Among 105 patients with UCTD (13.3% of total subjects), 44 had a UIP pattern (by surgical lung biopsy: 24; by high-resolution CT scan: 20), 29 had a nonspecific interstitial pneumonia pattern (by surgical lung biopsy), and nine had an organizing pneumonia pattern (by biopsy). The overall survival of the UCTD-UIP group was shorter than that of the UCTDNSIP group (P = .021) but significantly better than that of the IPF group (P = .042). Conclusions: A UIP pattern, which seems to be frequent in UCTD, showed a poorer prognosis than that of UCTD-NSIP. However, the prognosis of UCTD-UIP was significantly better than that of IPF, highlighting the importance of searching for underlying UCTD in suspected IPF cases.

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