Sjögren's syndrome (SS) has a varied clinical spectrum and has been associated with various chest computed tomography (CT) findings. We sought to delineate the characteristic CT features in various subsets of SS, especially poor prognosis subsets.
Retrospectively identified 80 never-smoker SS patients [56 primary SS (1-SS), 24 secondary SS (2-SS)] who underwent chest CT at our institution during a 3-year period from 2004 through 2007 were included in this study. Chest CT findings were qualitatively and semiquantitatively analyzed with comparison between 1-SS and 2-SS, and correlation with anti-SSB/La seropositivity and the presence of clonally derived lymphoproliferative disorder (cLPD), which are known to be pathognomonic and prognostic clinical features of SS patients.
All patients were women with median age of 60 years. Anti-SSB/La antibodies were found in 17 primary SS patients and 4 2-SS patients. Eleven patients with cLPD were identified and all of them had 1-SS. The most frequent CT finding in both types of patients was interlobular septal thickening. Secondary SS was associated with a significantly greater frequency and extent of honeycombing versus 1-SS. Univariate and multivariate analysis showed a significant association between honeycombing and 2-SS. In patients with 1-SS and in the SS group as a whole, we observed independent and significant associations between cysts and anti-SSB/La seropositivity or cLPD.
Cysts are significantly associated with anti-SSB/La seropositivity and cLPD. The presence of lung cysts revealed by chest CT might be a prognostic clinical feature, a clue, or a predictor of cLPD in patients with SS.