Existing longitudinal studies of persons with SS show that the clinical course of SS is slowly progressive and is life altering, rather than life threatening, for most patients. One study found no overall increased mortality in a population of individuals with SS when compared to controls (12). Those with SS also do not have increased cardiovascular mortality, as do individuals with SLE, despite the apparent overlap in signs and symptoms. Salivary glands in SS begin with focal lymphocytic infiltrates but can progress to a lymphoepithelial lesion (lymphoepithelial sialadenitis). This is often associated with chronic clinical enlargement of major salivary glands, occurring in about one-third of patients. Rarely, it may progress to a MALT lymphoma, an indolent tumor progressing for years, or become a high-grade B-cell non-Hodgkin's lymphoma. Various studies have shown a significantly higher prevalence of lymphoma in SS patients than in the general population. Lymphomas occur most frequently in the salivary glands, but also can occur in the gastrointestinal tract, thyroid gland, lung, kidney, lacrimal glands, or lymph nodes. Clinical predictors for lymphoma development in SS include low C4 level, persistently enlarged salivary glands, regional or general lymphadenopathy, hepatosplenomegaly, pulmonary infiltrates, vasculitis in a setting of hypergammaglobulinemia, high erythrocyte sedimentation rate associated with hypergammaglobulinemia, and a monoclonal immunoglobulinemia.
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