{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:15:08Z","timestamp":1792088108038,"version":"4.4.0"},"reference-count":49,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2005,3,1]],"date-time":"2005-03-01T00:00:00Z","timestamp":1109635200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,3,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjo\u0308gren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern. Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjo\u0308gren's syndrome in the light of NSIP, and to analyze prognosis of the disease. Methods: On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis. Measurements and main results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low PaO2 (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean \u00b1 SD: 68.5 \u00b1 16.6%pred) than patients without NSIP (92.5 \u00b1 18.6%pred; p &amp;lt; 0.001). Conclusion: Among a diversity of pulmonary lesions in primary Sjo\u0308gren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.<\/jats:p>","DOI":"10.1164\/rccm.200403-417oc","type":"journal-article","created":{"date-parts":[[2004,12,3]],"date-time":"2004-12-03T20:25:25Z","timestamp":1102105525000},"page":"632-638","source":"Crossref","is-referenced-by-count":303,"title":["Pulmonary Manifestations of Primary Sjo\u0308gren's Syndrome"],"prefix":"10.1093","volume":"171","author":[{"given":"Isao","family":"Ito","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonoko","family":"Nagai","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori","family":"Kitaichi","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew G.","family":"Nicholson","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Johkoh","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Noma","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dong Soon","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Handa","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takateru","family":"Izumi","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michiaki","family":"Mishima","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Laboratory of Anatomic Pathology, Kyoto University Hospital; Central Clinic in Kyoto, Kyoto; Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka; Department of Radiology, Tenri Hospital, Nara, Japan; Department of Histopathology, Royal Brompton Hospital, London, United Kingdom; and Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, 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