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Interstitial pneumonia related to undifferentiated connective tissue disease: pathologic pattern and prognosis.
Chest. 2015 Jan; 147(1):165-172.Chest

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 UCTD-NSIP 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.

Authors+Show Affiliations

Department of Pulmonary and Critical Care Medicine; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Department of Pulmonary and Critical Care Medicine; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Department of Radiology; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea; Department of Laboratory Medicine and Pathology; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Mayo Clinic, Scottsdale, AZ; Department of Pathology; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Division of Rheumatology, Department of Internal Medicine; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Department of Clinical Epidemiology and Biostatistics; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.Department of Pulmonary and Critical Care Medicine; Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea. Electronic address: dskim615@gmail.com.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

25211168

Citation

Kim, Ho-Cheol, et al. "Interstitial Pneumonia Related to Undifferentiated Connective Tissue Disease: Pathologic Pattern and Prognosis." Chest, vol. 147, no. 1, 2015, pp. 165-172.
Kim HC, Ji W, Kim MY, et al. Interstitial pneumonia related to undifferentiated connective tissue disease: pathologic pattern and prognosis. Chest. 2015;147(1):165-172.
Kim, H. C., Ji, W., Kim, M. Y., Colby, T. V., Jang, S. J., Lee, C. K., Han, S. B., & 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
Kim HC, et al. Interstitial Pneumonia Related to Undifferentiated Connective Tissue Disease: Pathologic Pattern and Prognosis. Chest. 2015;147(1):165-172. PubMed PMID: 25211168.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Interstitial pneumonia related to undifferentiated connective tissue disease: pathologic pattern and prognosis. AU - Kim,Ho-Cheol, AU - Ji,Wonjun, AU - Kim,Mi Young, AU - Colby,Thomas V, AU - Jang,Se Jin, AU - Lee,Chang-Keun, AU - Han,Seung Bong, AU - Kim,Dong Soon, PY - 2014/9/12/entrez PY - 2014/9/12/pubmed PY - 2015/3/11/medline SP - 165 EP - 172 JF - Chest JO - Chest VL - 147 IS - 1 N2 - 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 UCTD-NSIP 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. SN - 1931-3543 UR - https://www.unboundmedicine.com/medline/citation/25211168/Interstitial_pneumonia_related_to_undifferentiated_connective_tissue_disease:_pathologic_pattern_and_prognosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(15)30246-4 DB - PRIME DP - Unbound Medicine ER -