Objectives To investigate the prognostic value of the lymphocytic focus score (LFS) and the percentages of IgA+, IgM+ and IgG+ plasma cells for disease severity of primary Sjögren syndrome (pSS).
Methods Medical charts of 174 pSS patients were retrospectively analysed, comparing histology results (LFS and percentages of IgA+, IgM+ and IgG+ plasma cells) with disease outcomes as non-Hodgkin lymphoma (NHL) and clinical scores including cumulative EULAR (European League against Rheumatism) Sjögren syndrome disease activity index (ESSDAI) and the total number of extraglandular manifestations.
Results The mean LFS was significantly higher in patients developing NHL (3.0±0.894 vs 2.25±1.086; p=0.021). The threshold of ≥3 foci has a positive predictive value of 16% for lymphoma, and a negative predictive value of 98%. Only LFS ≥3 contributed significantly and independently to NHL development in a standard multiple regression model. Ig class distribution of plasma cells did not help to identify patients developing lymphoma. Patients with LFS ≥3, ≤40% IgA+ or ≥25% IgM+ plasma cells in salivary gland biopsy specimens had significantly enhanced systemic disease.
Conclusions Routine histopathological minor salivary gland assessment has important prognostic value. The LFS might help to identify patients with an increased risk for lymphoma.
- Sjøgren's Syndrome
- Outcomes Research
- Disease Activity
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