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Primary Sjögren’s syndrome is a systemic autoimmune disease characterised by chronic inflammation of exocrine glands, primarily the salivary and lacrimal glands. In the glands, ectopic lymphoid tissue may form, with germinal centre-like structures promoting B-cell DNA rearrangements and Ro/SSA and La/SSB autoantibody production.1 The presence of autoantibodies correlate with disease severity and influence long-term outcome.2 High circulating levels of BAFF also contributes to the polyclonal B-cell activation, and increased plasmablast differentiation and hypergammaglobulinaemia are common.3 4 The autoantibodies can induce production of type I interferons, which further a positive feed-forward loop of chronic B-cell activation.3
Patients with primary Sjögren’s syndrome have a 5–15 times higher risk of lymphoma than the general population, corresponding to a lifetime risk of 5%–10%.5 The malignancies are commonly B-cell non-Hodgkin lymphomas, predominantly marginal zone lymphomas. However, whether there is an increased risk of multiple myeloma in Sjögren’s syndrome has not been unequivocally defined (see online supplementary text).6 Considering the abundance and activity of …
Handling editor Josef S Smolen
Contributors JM, MW-H and GN conceived the study, with additional support from EB and KES. MK, HF-dE, SM-B, PE, TM and GN extracted register data. JM analysed the data with input from MW-H and GN. JM wrote the first manuscript with input from MW-H, GN and EB. All authors participated in the editing of the manuscript until its final version.
Funding The study was supported by grants from the Swedish Research Council, the Swedish Rheumatism Association, the King Gustaf the V's 80-year foundation, the Heart-Lung Foundation, the Freemason’s in Stockholm Foundation for Children’s Welfare, the Stockholm County Council, the Karolinska Institute and the Torsten and Ragnar Söderberg Foundation.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval The study was approved by the Regional Ethics Committee in Stockholm, and patients gave written informed consent.
Provenance and peer review Not commissioned; externally peer reviewed.