Risk of Non-Hodgkin's Lymphoma in Primary Sjögren's Syndrome: A Population-Based Study
Version of Record online: 23 APR 2013
Copyright © 2013 by the American College of Rheumatology
Arthritis Care & Research
Volume 65, Issue 5, pages 816–821, May 2013
How to Cite
Johnsen, S. J., Brun, J. G., Gøransson, L. G., Småstuen, M. C., Johannesen, T. B., Haldorsen, K., Harboe, E., Jonsson, R., Meyer, P. A. and Omdal, R. (2013), Risk of Non-Hodgkin's Lymphoma in Primary Sjögren's Syndrome: A Population-Based Study. Arthritis Care Res, 65: 816–821. doi: 10.1002/acr.21887
- Issue online: 23 APR 2013
- Version of Record online: 23 APR 2013
- Accepted manuscript online: 8 NOV 2012 01:13PM EST
- Manuscript Accepted: 11 OCT 2012
- Manuscript Received: 22 MAR 2012
- Broegelmann Foundation (University of Bergen)
- Norwegian Rheumatism Association
- Norwegian Women's Public Health Association
- Western Norway Regional Health Authority
Primary Sjögren's syndrome (SS) is associated with an increased risk of non-Hodgkin's lymphoma (NHL), but the reported prevalence and risk vary considerably. The objective of this study was to determine the risk of NHL in a well-defined population-based primary SS cohort in Norway.
The authors examined all patients fulfilling the American–European Consensus Group criteria for primary SS from 2 Norwegian counties and compared the data to the Cancer Registry of Norway to identify the primary SS patients who had lymphoma. In addition, lymphoma patient files from the same period were reviewed for undiagnosed primary SS to ensure the quality of registry data.
As of July 1, 2009, 443 living subjects with primary SS were identified in an area with 896,840 inhabitants, which is 18.6% of the total population of Norway. Seven cases of NHL (1.6%) were found during a total followup of 3,813 person-years, resulting in a standardized incidence ratio of 9.0 (95% confidence interval 7.1–26.3) for NHL in primary SS patients.
The risk of NHL in patients with primary SS in Norway is increased 9 times compared with the general population. This is in accordance with recent studies, and the quality and completeness of the registries and strict use of diagnostic criteria support the validity of the results.