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For most of us, primary Sjogren's Syndrome (pSS) remains a challenging disease to manage. There have been very few treatments proven to be effective and those that may be effective are often difficult to access. We know that patients with pSS are at higher risk of developing non-Hodgkin lymphoma (NHL), but we have very few clinical tools to predict its development.
In one study, patients meeting criteria for pSS had a 16-fold higher risk of developing NHL than those who did not meet criteria (Theander, ARD 2006). Furthermore, the risk of developing lymphoma has been shownto increase over time. The presence of an ectopic germinal centre on biopsy of the salivary glands can predict the development of NHL in patients with pSS. However, glandular biopsies are invasive and are therefore not practical for long-term patient follow-up. Clinically, parotid enlargement, palpable purpura, low C4, and CD4+ T-cell lymphocytopenia (Dong, 2013) may be indicators of lymphoma development, but these have not been well studied. Many of us use physical exam and periodic lab screening (including CBC, SPEP, andcomplement) when following our patients.
Gaetane Nocturne presented data from her group in France, evaluating if there is an association between levels of the chemokines CCL11 and CXCL13 (both of which have been associated with ectopic germinal centres) and NHL in patients with pSS in the ASSESS cohort. Of the 385 patients with pSS in the ASSESS cohort, 22 had a previous diagnosis of NHL and five developed NHL ("future lymphoma," i.e. after chemokine levels were drawn). Patients with previous or future lymphoma had significantly higher levels of CXCL13 compared to those without lymphoma (p=0.006). There was a trend for an association between CCL11 and NHL (p=0.056). These associations were highest in the five patients who developed lymphoma after serum chemokine levels were drawn. There was also a statistically significant correlation between chemokine levels and Sjogren's disease activity based on the Clin ESSDAI score (CCL11 and Clin ESSDAI).
In a multivariate analysis of other associations with lymphoma, low C4 (p=0.04) and low BAFF levels (p=0.0002) were statistically significant. This has been reported in other studies.
What does this mean to clinicians? There is likely an association between Sjogren's disease activity, duration, and development of NHL. Although we all know that this risk needs to be monitored, there are no clear guidelines on how best to do this. Chemokines show promise for the future.
This study was the first step and identified an association with NHL, but more work is needed to determine if chemokines can be predictive and if so, commercialization of assays may provide clinical tools in the future. In the meantime, we should continue to rely on history, physical exam and basic lab monitoring with repeat biopsy looking for ectopic germinal centres in high-risk patients.
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About the Author
Dr. Shahin Jamal
Dr. Jamal is a Clinical Associate Professor at the University of British Columbia and an active staff physician at Vancouver Coastal Health. Her interests include diagnosis and prognosis of early inflammatory arthritis, and timely assessment and access to care for patients with rheumatoid arthritis.
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