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Abstract
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.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.
No writing assistance was utilized in the production of this manuscript.
Key issues
Indolent marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type has been identified by several studies as the most common lymphoma subtype in primary Sjögren's syndrome (pSS).
Recent studies report a predominance of diffuse large B-cell lymphomas in pSS, implying that pSS-related non-Hodgkin's lymphomas is not subtype specific.
The risk of lymphoma development is maintained at high levels even 10 years after pSS diagnosis.
Patients presenting with swelling of the parotid gland, vasculitic purpura, splenomegaly, lymphadenopathy, cryoglobulinemia, low C4 levels, neutropenia and lymphopenia at diagnosis have a much greater probability of developing lymphoma and are considered as high-risk patients.
Lymphocytopenia at diagnosis can be used as a predictor of the development of a non-marginal zone B-cell lymphoma, mainly diffuse large B-cell lymphoma.
B-cell activating factor may represent a promising biologic marker of increased risk of lymphoproliferation in pSS.
Immunophenotyping studies indicate disturbances in the distribution of various peripheral B-cell subpopulations in pSS.
pSS patients with cryoglobulinemia and/or overt lymphoma displayed significantly higher percentages of CD27-CD21-/low B cells than those without such complications.
Elevated Flt-3L serum levels correlated with lymphoma development in pSS patients.