title: Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis.
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).
  
  
  CONCLUSIONS
  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.affiliation: 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.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Enomoto
authors.affiliation: 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.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.affiliation: 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.
authors.forename: Eri
authors.initials: E
authors.lastname: Hagiwara
authors.affiliation: 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.
authors.forename: Tae
authors.initials: T
authors.lastname: Iwasawa
authors.affiliation: 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.
authors.forename: Koji
authors.initials: K
authors.lastname: Okudela
authors.affiliation: 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.
authors.forename: Noriyo
authors.initials: N
authors.lastname: Yanagawa
authors.affiliation: 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.
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Baba
authors.affiliation: Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, 1298, Yamane, Hidaka, Saitama 350-1298, Japan. Electronic address: fmksakai@yahoo.co.jp.
authors.forename: Fumikazu
authors.initials: F
authors.lastname: Sakai
authors.affiliation: Department of Diagnostic Pathology, Itabashi Chuo Medical Center, 2-12-7, Azusawa, Itabashi-ku, Tokyo 174-0051, Japan. Electronic address: fukuda@nms.ac.jp.
authors.forename: Yuh
authors.initials: Y
authors.lastname: Fukuda
authors.affiliation: Department of Rheumatology, Yokohama Minami Kyosai Hospital, 1-21-1, Mutsuura-Higashi, Kanazawa-ku, Yokohama 236-0037, Japan. Electronic address: nagaokascrt@msn.com.
authors.forename: Shouhei
authors.initials: S
authors.lastname: Nagaoka
authors.affiliation: 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.
authors.forename: Takashi
authors.initials: T
authors.lastname: Ogura
mesh_terms: D000367:Age Factors; D000368:Aged; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012737:Sex Factors; D012859:Sjogren's Syndrome / Q000150:complications*; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Idiopathic pulmonary fibrosis; Immunosuppressive treatment; Sjögren׳s syndrome; Usual interstitial pneumonia