Abstract

Patients with Sjögren's disease (pSS) are at increased risk of lymphoid hemopathy. The development of lymphoma is the most serious complication of SJS. It occurs in 5 to 10% of the patients. Lymphomas in pSS are most often extra-nodal marginal zone non-Hodgkin's B-cell lymphomas that develop from the mucosa associated lymphoid tissue. They frequently occur in the salivary glands, which are the target organ of this autoimmune disease. The development of lymphoma in pSS is a multistep process related to chronic antigenic stimulation of B cells. This update reviews the main predictive factors for lymphoma in pSS and also the practical diagnostic and therapeutic management. In addition, it reviews the risk of myeloma in pSS, a much lesser known complication that is almost as common as lymphoma.

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