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Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias.
Respir Med. 2015 Mar; 109(3):389-96.RM

Abstract

BACKGROUND

Kinder et al. proposed a broader definition of undifferentiated connective tissue disease (UCTD) and reported that the entity of nonspecific interstitial pneumonia (NSIP) is a lung manifestation of this more broadly defined UCTD. However, a retrospective study did not support their findings and its clinical significance remains unclear.

METHODS

We prospectively evaluated the significance of this broadly defined UCTD in idiopathic interstitial pneumonias (IIPs) in consecutive patients with surgical lung biopsy. Patients were evaluated with a symptoms check list and underwent comprehensive serologic testing as screening for UCTD. Clinical characteristics, high-resolution CT images, lung biopsy specimens, serial FVC change, and survival were analyzed.

RESULTS

Among 76 patients with IIPs, 24 patients (32%) fulfilled the UCTD criteria. Diagnosis of 24 patients with UCTD was usual interstitial pneumonia in 12 (50%), NSIP in 7 (29%), and unclassifiable interstitial lung disease (ILD) in 5 (21%). The diagnosis of 52 patients who did not have UCTD was idiopathic pulmonary fibrosis in 27 (52%), NSIP in 11 (21%), unclassifiable ILD in 13 (25%) and cryptogenic organizing pneumonia in 1 (2%). One-year and two-year FVC changes showed no significant difference between UCTD and non-UCTD, however, significant differences in FVC change were observed among histopathological diagnoses both in UCTD and in non-UCTD. In multivariate survival analysis, %FVC and histopathological UIP pattern were independent predictors for survival but UCTD diagnosis was not.

CONCLUSIONS

A diagnosis of UCTD was not useful in discriminating NSIP or in predicting disease progression and prognosis in our cohort of IIPs. Histopathological UIP pattern was an independent predictor for mortality irrespective of a diagnosis of UCTD.

Authors+Show Affiliations

Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan. Electronic address: konyasu2003@yahoo.co.jp.Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Japan.Department of Laboratory of Pathology, Nagasaki University Hospital, Japan.Department of Radiology, Dokkyo University School of Medicine, Japan.Department of Laboratory of Pathology, Nagasaki University Hospital, Japan.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan.Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan.Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan.Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan.

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

25682542

Citation

Kondoh, Yasuhiro, et al. "Broader Criteria of Undifferentiated Connective Tissue Disease in Idiopathic Interstitial Pneumonias." Respiratory Medicine, vol. 109, no. 3, 2015, pp. 389-96.
Kondoh Y, Johkoh T, Fukuoka J, et al. Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias. Respir Med. 2015;109(3):389-96.
Kondoh, Y., Johkoh, T., Fukuoka, J., Arakawa, H., Tanaka, T., Watanabe, N., Sakamoto, K., Kataoka, K., Kimura, T., & Taniguchi, H. (2015). Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias. Respiratory Medicine, 109(3), 389-96. https://doi.org/10.1016/j.rmed.2015.01.009
Kondoh Y, et al. Broader Criteria of Undifferentiated Connective Tissue Disease in Idiopathic Interstitial Pneumonias. Respir Med. 2015;109(3):389-96. PubMed PMID: 25682542.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Broader criteria of undifferentiated connective tissue disease in idiopathic interstitial pneumonias. AU - Kondoh,Yasuhiro, AU - Johkoh,Takeshi, AU - Fukuoka,Junya, AU - Arakawa,Hiroaki, AU - Tanaka,Tomonori, AU - Watanabe,Naohiro, AU - Sakamoto,Koji, AU - Kataoka,Kensuke, AU - Kimura,Tomoki, AU - Taniguchi,Hiroyuki, Y1 - 2015/01/31/ PY - 2014/07/26/received PY - 2014/11/30/revised PY - 2015/01/22/accepted PY - 2015/2/16/entrez PY - 2015/2/16/pubmed PY - 2016/2/9/medline KW - IIPs – idiopathic interstitial pneumonias KW - IPF – idiopathic pulmonary fibrosis KW - NSIP – nonspecific interstitial pneumonia KW - SLB – surgical lung biopsy KW - UCTD – undifferentiated connective tissue disease SP - 389 EP - 96 JF - Respiratory medicine JO - Respir Med VL - 109 IS - 3 N2 - BACKGROUND: Kinder et al. proposed a broader definition of undifferentiated connective tissue disease (UCTD) and reported that the entity of nonspecific interstitial pneumonia (NSIP) is a lung manifestation of this more broadly defined UCTD. However, a retrospective study did not support their findings and its clinical significance remains unclear. METHODS: We prospectively evaluated the significance of this broadly defined UCTD in idiopathic interstitial pneumonias (IIPs) in consecutive patients with surgical lung biopsy. Patients were evaluated with a symptoms check list and underwent comprehensive serologic testing as screening for UCTD. Clinical characteristics, high-resolution CT images, lung biopsy specimens, serial FVC change, and survival were analyzed. RESULTS: Among 76 patients with IIPs, 24 patients (32%) fulfilled the UCTD criteria. Diagnosis of 24 patients with UCTD was usual interstitial pneumonia in 12 (50%), NSIP in 7 (29%), and unclassifiable interstitial lung disease (ILD) in 5 (21%). The diagnosis of 52 patients who did not have UCTD was idiopathic pulmonary fibrosis in 27 (52%), NSIP in 11 (21%), unclassifiable ILD in 13 (25%) and cryptogenic organizing pneumonia in 1 (2%). One-year and two-year FVC changes showed no significant difference between UCTD and non-UCTD, however, significant differences in FVC change were observed among histopathological diagnoses both in UCTD and in non-UCTD. In multivariate survival analysis, %FVC and histopathological UIP pattern were independent predictors for survival but UCTD diagnosis was not. CONCLUSIONS: A diagnosis of UCTD was not useful in discriminating NSIP or in predicting disease progression and prognosis in our cohort of IIPs. Histopathological UIP pattern was an independent predictor for mortality irrespective of a diagnosis of UCTD. SN - 1532-3064 UR - https://www.unboundmedicine.com/medline/citation/25682542/Broader_criteria_of_undifferentiated_connective_tissue_disease_in_idiopathic_interstitial_pneumonias_ DB - PRIME DP - Unbound Medicine ER -