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Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis.
Respir Investig. 2014 Jul; 52(4):227-35.RI

Abstract

BACKGROUND

The different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients.

METHODS

Among 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation.

RESULTS

More patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addition, they more commonly exhibited enlarged mediastinal lymph nodes and bronchial wall thickening on HRCT. Pathologically, in the UIP/pSS group, interstitial inflammation, plasma cell infiltration, lymphoid follicles with germinal centers, cysts, bronchiolitis, and pleuritis were significantly more prominent, whereas smooth muscle hyperplasia and fibroblastic foci were milder (all P<0.05). The prognosis was better for UIP/pSS compared with UIP/IPF patients (P=0.01). In addition, immunosuppressive therapy provided better disease control for those with UIP/pSS (83%, 5/6) compared UIP/IPF (7%, 1/15).

CONCLUSION

This study identified distinct clinical, radiological, and pathological characteristics of UIP/pSS compared with UIP/IPF. Immunosuppressive treatment could be a therapeutic option for UIP/pSS.

Authors+Show Affiliations

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: yasuyasuyasu29@yahoo.co.jp.Department of Pathology, Japanese Red Cross Medical Center, 4-1-22, Hiroo, Shibuya-ku, Tokyo 150-8935, Japan. Electronic address: byori@med.jrc.or.jp.Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: hagiwara@kanagawa-junko.jp.Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: tae_i_md@wb3.so-net.ne.jp.Department of Pathobiology, Yokohama City University Graduate School of Medicine, 3-9, Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan. Electronic address: krascv@yahoo.co.jp.Department of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-18-22, Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. Electronic address: noriyo_yana@ybb.ne.jp.Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: baba@kanagawa-junko.jp.Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, 1298, Yamane, Hidaka, Saitama 350-1298, Japan. Electronic address: fmksakai@yahoo.co.jp.Department of Diagnostic Pathology, Itabashi Chuo Medical Center, 2-12-7, Azusawa, Itabashi-ku, Tokyo 174-0051, Japan. Electronic address: fukuda@nms.ac.jp.Department of Rheumatology, Yokohama Minami Kyosai Hospital, 1-21-1, Mutsuura-Higashi, Kanazawa-ku, Yokohama 236-0037, Japan. Electronic address: nagaokascrt@msn.com.Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address: takashiogurajunko@yahoo.co.jp.

Pub Type(s)

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

Language

eng

PubMed ID

24998369

Citation

Enomoto, Yasunori, et al. "Features of Usual Interstitial Pneumonia in Patients With Primary Sjögren׳s Syndrome Compared With Idiopathic Pulmonary Fibrosis." Respiratory Investigation, vol. 52, no. 4, 2014, pp. 227-35.
Enomoto Y, Takemura T, Hagiwara E, et al. Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis. Respir Investig. 2014;52(4):227-35.
Enomoto, Y., Takemura, T., Hagiwara, E., Iwasawa, T., Okudela, K., Yanagawa, N., Baba, T., Sakai, F., Fukuda, Y., Nagaoka, S., & Ogura, T. (2014). Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis. Respiratory Investigation, 52(4), 227-35. https://doi.org/10.1016/j.resinv.2014.02.003
Enomoto Y, et al. Features of Usual Interstitial Pneumonia in Patients With Primary Sjögren׳s Syndrome Compared With Idiopathic Pulmonary Fibrosis. Respir Investig. 2014;52(4):227-35. PubMed PMID: 24998369.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis. AU - Enomoto,Yasunori, AU - Takemura,Tamiko, AU - Hagiwara,Eri, AU - Iwasawa,Tae, AU - Okudela,Koji, AU - Yanagawa,Noriyo, AU - Baba,Tomohisa, AU - Sakai,Fumikazu, AU - Fukuda,Yuh, AU - Nagaoka,Shouhei, AU - Ogura,Takashi, Y1 - 2014/04/13/ PY - 2014/01/22/received PY - 2014/02/18/revised PY - 2014/02/23/accepted PY - 2014/7/8/entrez PY - 2014/7/8/pubmed PY - 2015/8/19/medline KW - Idiopathic pulmonary fibrosis KW - Immunosuppressive treatment KW - Sjögren׳s syndrome KW - Usual interstitial pneumonia SP - 227 EP - 35 JF - Respiratory investigation JO - Respir Investig VL - 52 IS - 4 N2 - BACKGROUND: The different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients. METHODS: Among 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation. RESULTS: More patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addition, they more commonly exhibited enlarged mediastinal lymph nodes and bronchial wall thickening on HRCT. Pathologically, in the UIP/pSS group, interstitial inflammation, plasma cell infiltration, lymphoid follicles with germinal centers, cysts, bronchiolitis, and pleuritis were significantly more prominent, whereas smooth muscle hyperplasia and fibroblastic foci were milder (all P<0.05). The prognosis was better for UIP/pSS compared with UIP/IPF patients (P=0.01). In addition, immunosuppressive therapy provided better disease control for those with UIP/pSS (83%, 5/6) compared UIP/IPF (7%, 1/15). CONCLUSION: This study identified distinct clinical, radiological, and pathological characteristics of UIP/pSS compared with UIP/IPF. Immunosuppressive treatment could be a therapeutic option for UIP/pSS. SN - 2212-5353 UR - https://www.unboundmedicine.com/medline/citation/24998369/Features_of_usual_interstitial_pneumonia_in_patients_with_primary_Sjögren׳s_syndrome_compared_with_idiopathic_pulmonary_fibrosis_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2212-5345(14)00024-0 DB - PRIME DP - Unbound Medicine ER -