Prognosis

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:

• Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, Granata G, Ng WF, Fisher BA, Bombardieri M, Astorri E, Empson B, Larkin G, Crampton B, Bowman SJ., British Society for Rheumatology Standards, Guideline and Audit Working Group. The British Society for Rheumatology guideline for the management of adults with primary Sjogren's Syndrome. Rheumatology (Oxford). 2017 Oct 01;56(10):e24-e48