Prognostic Value Of Minor Salivary Gland Assessments In Primary Sjögren’s Syndrome

Anna Risselada¹, A.a. Kruize², J.A.G. van Roon³, F.P.J.G. Lafeber⁴ and J.W.J. Bijlsma⁴, ¹Clinical immunology and Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands, ²Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ³Rheumatology & Clinical Immunology/Lab Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁴Rheumatology & Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Disease Activity, histopathologic and salivary gland, Sjogren's syndrome

Session Information

Title: Sjögren's Syndrome: Clinical Advances

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Salivary gland assessment is important for diagnosing primary Sjögren’s syndrome (pSS), as the lymphocytic focus score (LFS) is part of pSS classification criteria. Quantitative immunohistology (QIH) is another technique used for assessing pSS in which the percentage of immunoglobulin-specific plasma cells is measured, with IgA <70% and/or IgM >10% as positive criteria. Our objective was to investigate the prognostic value of minor salivary gland assessment (LFS and QIH) for disease severity of pSS.

Methods:

Medical charts of all patients with pSS according to the 2002 classification criteria who attended our outpatient clinic for at least one year from 1998 to July 2011 were analyzed. In total, 174 patients had documented minor salivary gland histology and were included for this analysis. Histology results (LFS and percentages of IgA, IgM and IgG plasma cells) were compared with disease outcomes as non-Hodgkin lymphoma (NHL) and clinical scores: cumulative EULAR Sjögren’s Syndrome disease activity index (ESSDAI) and the number of extraglandular manifestations (EGM) during disease course.

Results:

Mean age at pSS diagnosis was 47 ± 14 years and median follow-up after biopsy was 105 months (range 10 – 408). LFS ≥1 were seen in 99%, <70% IgA-positive plasma cells in 90%, and >10% IgM-positive plasma cells in 71% of patients, respectively. The number of foci correlated to a decrease in the percentage of IgA-positive plasma cells (R=0.315, p=0.0001), but not to the percentage of IgM- or IgG-positive plasma cells.

NHL developed in 16 patients (9%). The mean LFS was significantly higher in patients with NHL (3.0 ± 0.9 versus 2.3 ± 1.1; p=0.021). The threshold of ≥3 foci had a positive predictive value of 16% for development of lymphoma, and a negative predictive value of 98% (OR 7.9; p=0,008). QIH results could not predict lymphoma development.

Only LFS ≥3 contributed significantly and independently to NHL development in a hierarchical multiple regression model, correcting for presence of anti-SSA/SSB antibodies (beta 0.244; p=0.017). Cumulative ESSDAI and EGM were significantly correlated to LFS, a decreased percentage of IgA-positive and an increased percentage of IgM-positive plasma cells (R range 0.166 – 0.284; p≤0.04), but not to the percentage of IgG-positive plasma cells.

Conclusion:

Routinely performed minor salivary gland assessments have important prognostic value. The number of lymphocyte foci can be used to identify patients with increased lymphoma risk.

Acknowledgements:

We would like to thank J.M. van Woerkom, R. Goldschmeding and the pathology department of the University Medical Center Utrecht for their work on the minor salivary gland assessments.

Disclosure:

A. Risselada,
None;

A. A. Kruize,
None;

J. A. G. van Roon,
None;

F. P. J. G. Lafeber,
None;

J. W. J. Bijlsma,
None.

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