Abstract
Background: Although pulmonary involvement of Sjögren's syndrome (SS) has been reported, its clinical characteristics have not been clear in detail. The goal of this study was to clarify the characteristics of pulmonary manifestation in patients with SS.
Methods: We reviewed patient with SS admitted to our hospital from 2007 to 2011, and retrospective analysis was performed.
Results: We had 37 patients with SS including 26 primary (pSS) and 11 secondary Sjögren's syndrome (sSS). Only 11 patients had mucous membrane drying symptoms. Anti-SSA and anti-SSB antibody in serum were positive in 32 and 13 patients, respectively. 33 patients had consistent findings of fluorescent dye examination and/or lip biopsy with SS. Interstitial pneumonia (IP) was the main pulmonary manifestation. In fact, 10.2% of IP patients with unknown etiology on admission were diagnosed as having SS by a careful clinical evaluation. Histopathological evaluation revealed a variety of histological findings such as non-specific IP, and cellular bronchiolitis. Grand glass attenuation was the major HRCT pattern with lower and peripheral predominant distributions, and analysis of bronchoalveolar lavage fluid showed increase number of lymphocytes and neutrophils. Five-year survival rate was about 80%, and prognosis in patients with pSS seemed to be better than that in sSS patients.
Conclusions: IP is the major pulmonary manifestation of SS, and prognosis is not so poor. For more understanding of this disease, a careful clinical evaluation including lip biopsy is necessary for IP patients even without sicca symptoms because of under-diagnosis of IP patients with SS.
- © 2013 ERS
