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Increased risk of multiple myeloma in primary Sjögren’s syndrome is limited to individuals with Ro/SSA and La/SSB autoantibodies
  1. Johannes Mofors1,
  2. Albin Björk1,
  3. Karin E Smedby2,
  4. Marika Kvarnström1,
  5. Helena Forsblad-d'Elia3,
  6. Sara Magnusson-Bucher4,
  7. Per Eriksson5,
  8. Thomas Mandl6,
  9. Eva Baecklund7,
  10. Gunnel Nordmark7,
  11. Marie Wahren-Herlenius1
  1. 1Division of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
  2. 2Division of clinical epidemiology, Department of Medicine Solna, Karolinska Institutet, and Department of Hematology, Karolinska University hospital, Stockholm, Sweden
  3. 3Department of Public Health and Clinical Medicine, Rheumatology, Umeå University, Umeå, Sweden
  4. 4Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden
  5. 5Rheumatology/Division of Neuro and Inflammation Sciences, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden
  6. 6Department of Clinical Sciences, Malmö, Rheumatology, Lund University, Malmö, Sweden
  7. 7Department of Medical Sciences, Rheumatology and Science for Life Laboratory, Uppsala University, Uppsala, Sweden
  1. Correspondence to Professor Marie Wahren-Herlenius, Department of Medicine, Karolinska Institutet, Stockholm SE-171 76, Sweden; marie.wahren{at}

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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 …

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