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Prognosis

Authoring team

Patients may develop develop a B cell lymphoma which may be predicted by circulating monoclonal cryoglobulins.

  • risk of lymphoma development in patients with primary SS increases from 3% in the first 5 years to 9.8% at 15 years, that is, up to 40 times the population risk (1)
  • presence of lymphadenopathy, parotid enlargement, palpable purpura, low serum C4 levels and cryoglobulins are the most consistent predictors of future risk
  • majority of lymphomas are of the mucosa-associated lymphoid tissue type
  • median age of onset is mid-50s and the diagnosis of Sjogren’s generally pre-dates the lymphoma by a mean of 7 years

One percent develop another autoimmune connective tissue disease.

Reference:


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