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Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features.
Chest. 2015 Dec; 148(6):1438-1446.Chest

Abstract

BACKGROUND

Lung-dominant connective tissue disease (LD-CTD) is a disease concept for interstitial pneumonia; however, it has not been robustly validated. This study was conducted to elucidate the clinical, radiologic, and histologic features of LD-CTD.

METHODS

We retrospectively reviewed 44 consecutive patients with serologically defined LD-CTD who underwent surgical lung biopsy. Patients were identified as having LD-CTD if they had specific autoantibodies but did not meet the criteria for connective tissue disease. We conducted a multidisciplinary diagnosis and evaluated major histologic patterns according to the current idiopathic interstitial pneumonias (IIPs) classification of 2013. Characteristic histologic features for LD-CTD (eg, prominent plasmacytic infiltration, lymphoid aggregates with germinal centers), high-resolution CT (HRCT) scan patterns, and prognosis were also assessed.

RESULTS

The major histologic patterns were usual interstitial pneumonia (UIP) in 25 patients and nonspecific interstitial pneumonia (NSIP) in 13 patients. Two or more characteristic histologic features for LD-CTD were observed in 15 patients with histologic UIP (h-UIP) and 11 patients with histologic NSIP (h-NSIP). Fifteen patients with h-UIP (60%) showed an inconsistent UIP pattern on HRCT scan. After multidisciplinary discussion (MDD), 18 patients with h-UIP were labeled as having unclassifiable IIP. The annual change in percent predicted FVC improved significantly in patients with h-NSIP (P = .002), who also had better survival than those with h-UIP (P = .031). In contrast, survival was not associated with HRCT scan pattern (P = .79).

CONCLUSIONS

The major histologic patterns in LD-CTD were UIP followed by NSIP. Two-thirds of patients had characteristic histologic features for LD-CTD. A majority of patients with h-UIP were considered to have unclassifiable IIP based on MDD. Patients with h-UIP had worse survival than those with h-NSIP.

Authors+Show Affiliations

Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi. Electronic address: taniguchi@tosei.or.jp.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi.Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi.Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Itami, Hyogo.Department of Radiology, Kurume University School of Medicine, Kurume, Fukuoka.Department of Laboratory of Pathology, Nagasaki University Hospital, Nagasaki, Nagasaki.Department of Diagnostic Pathology, Kobe University Hospital, Kobe, Hyogo.Department of Respiratory Medicine and Allergology, Kinki University Faculty of Medicine, Osaka-sayama, Osaka, Japan.Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi.

Pub Type(s)

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

Language

eng

PubMed ID

25950648

Citation

Omote, Norihito, et al. "Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features." Chest, vol. 148, no. 6, 2015, pp. 1438-1446.
Omote N, Taniguchi H, Kondoh Y, et al. Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features. Chest. 2015;148(6):1438-1446.
Omote, N., Taniguchi, H., Kondoh, Y., Watanabe, N., Sakamoto, K., Kimura, T., Kataoka, K., Johkoh, T., Fujimoto, K., Fukuoka, J., Otani, K., Nishiyama, O., & Hasegawa, Y. (2015). Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features. Chest, 148(6), 1438-1446. https://doi.org/10.1378/chest.14-3174
Omote N, et al. Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features. Chest. 2015;148(6):1438-1446. PubMed PMID: 25950648.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Lung-Dominant Connective Tissue Disease: Clinical, Radiologic, and Histologic Features. AU - Omote,Norihito, AU - Taniguchi,Hiroyuki, AU - Kondoh,Yasuhiro, AU - Watanabe,Naohiro, AU - Sakamoto,Koji, AU - Kimura,Tomoki, AU - Kataoka,Kensuke, AU - Johkoh,Takeshi, AU - Fujimoto,Kiminori, AU - Fukuoka,Junya, AU - Otani,Kyoko, AU - Nishiyama,Osamu, AU - Hasegawa,Yoshinori, PY - 2015/5/8/entrez PY - 2015/5/8/pubmed PY - 2016/4/28/medline SP - 1438 EP - 1446 JF - Chest JO - Chest VL - 148 IS - 6 N2 - BACKGROUND: Lung-dominant connective tissue disease (LD-CTD) is a disease concept for interstitial pneumonia; however, it has not been robustly validated. This study was conducted to elucidate the clinical, radiologic, and histologic features of LD-CTD. METHODS: We retrospectively reviewed 44 consecutive patients with serologically defined LD-CTD who underwent surgical lung biopsy. Patients were identified as having LD-CTD if they had specific autoantibodies but did not meet the criteria for connective tissue disease. We conducted a multidisciplinary diagnosis and evaluated major histologic patterns according to the current idiopathic interstitial pneumonias (IIPs) classification of 2013. Characteristic histologic features for LD-CTD (eg, prominent plasmacytic infiltration, lymphoid aggregates with germinal centers), high-resolution CT (HRCT) scan patterns, and prognosis were also assessed. RESULTS: The major histologic patterns were usual interstitial pneumonia (UIP) in 25 patients and nonspecific interstitial pneumonia (NSIP) in 13 patients. Two or more characteristic histologic features for LD-CTD were observed in 15 patients with histologic UIP (h-UIP) and 11 patients with histologic NSIP (h-NSIP). Fifteen patients with h-UIP (60%) showed an inconsistent UIP pattern on HRCT scan. After multidisciplinary discussion (MDD), 18 patients with h-UIP were labeled as having unclassifiable IIP. The annual change in percent predicted FVC improved significantly in patients with h-NSIP (P = .002), who also had better survival than those with h-UIP (P = .031). In contrast, survival was not associated with HRCT scan pattern (P = .79). CONCLUSIONS: The major histologic patterns in LD-CTD were UIP followed by NSIP. Two-thirds of patients had characteristic histologic features for LD-CTD. A majority of patients with h-UIP were considered to have unclassifiable IIP based on MDD. Patients with h-UIP had worse survival than those with h-NSIP. SN - 1931-3543 UR - https://www.unboundmedicine.com/medline/citation/25950648/Lung_Dominant_Connective_Tissue_Disease:_Clinical_Radiologic_and_Histologic_Features_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(15)50107-4 DB - PRIME DP - Unbound Medicine ER -