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Pulmonary Manifestations of Primary Sjögren's Syndrome

A Clinical, Radiologic, and Pathologic Study

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

Correspondence and requests for reprints should be addressed to Sonoko Nagai, M.D., Ph.D., Department of Respiratory Medicine, Kyoto University Hospital, 54 Shogoin-kawaharacho, Sakyo, Kyoto, Japan. E-mail: nagai{at}kuhp.kyoto-u.ac.jp

Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjögren'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 Sjögren'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 Pa_O2 (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean ± SD: 68.5 ± 16.6%pred) than patients without NSIP (92.5 ± 18.6%pred; p < 0.001). Conclusion: Among a diversity of pulmonary lesions in primary Sjögren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.

Key Words: bronchiolitis • honeycombing • nonspecific interstitial pneumonia • surgical lung biopsy • survival

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