Natural History

Few prospective studies have been performed in primary SS and, consequently, the natural history is poorly understood. The evidence obtained in cross-sectional studies suggests that multiple etiologic factors are involved and that the pathogenesis of this disorder is a multistage process fundamentally involving immunological mechanisms. Moreover, it is assumed that when progression occurs it is a slow process, clinically characterized by gradually increasing symptoms and signs. Within the immune system, there is a transition from polyclonal to monoclonal lymphocyte proliferation resulting in B-cell lymphoma, but this is applicable to only a few patients.

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