Predicting progression to lymphoma in Sjögren's syndrome patients

Although the inherent complexity of the multifactorial nature of primary Sjögren's syndrome (pSS) renders the process of disease prognostication and prediction ambiguous, certain clinical and immunological characteristics have been described as lymphoma predictors in several studies. While the association between pSS and mucosa-associated lymphoid tissue lymphomas is indisputable, recent studies report a predominance of diffuse large B-cell lymphomas implying that pSS-lymphoma association is less subtype-specific than previously considered. The considerable differences in both disease severity and prognosis between patients with various types of lymphoma demand the identification of risk factors that can predict the development of the distinct subtypes. Additionally, a recently discovered diverse range of biological variables appears to influence clinical behavior and lymphoma outcome. In this review, we venture into the area of lymphoma prognostication in pSS, outlining long-established predictors, analyzing currently available prognostic models, and exploring the predictive potential of recent biological and molecular advances.