Abstract
Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease mainly affecting exocrine glands and leading to impaired secretory function. The clinical picture is dominated by signs and symptoms of mucosal dryness and the course of the disease is mild and indolent in the majority of cases. However, a subgroup of patients can also experience extraglandular manifestations that worsen the disease prognosis. pSS patients are consistently found to have a higher risk of developing non-Hodgkin lymphoma (NHL) compared with patients with other autimmune disorders and to the general population. NHL is the most severe comorbidity that can occur in pSS, therefore recent research has aimed to identify reliable clinical, serological, and histological biomarkers able to predict NHL development in these subjects. This review article encompasses the body of evidence published so far in this field highlighting the challenges and pitfalls of different biomarkers within clinical practice. We also provide an overview of epidemiological data, diagnostic procedures, and evidence-based treatment strategies for NHL in pSS.
Highlights
Primary Sjögren’s syndrome is an autoimmune disorder primarily characterized by chronic inflammation of exocrine glands, leading to reduced secretory capacity [1, 2]
Non-Hodgkin’s lymphomas (NHL) occur in approximately 2.7–9.8% of Primary Sjögren’s syndrome (pSS) patients and recent data reported that non-Hodgkin lymphoma (NHL) risk increases 2.2% per year of age with a 4.3-fold increased risk in pSS compared with the general population
Over 70% of pSS-related mucosa-associated lymphoid tissue (MALT) lymphomas display mutations potentially leading to functional abnormalities in A20
Summary
Primary Sjögren’s syndrome (pSS) is an autoimmune disorder primarily characterized by chronic inflammation of exocrine glands, leading to reduced secretory capacity [1, 2]. These observations support a scenario in which the presence of germinal and/ or somatic abnormalities of genes, leading to impaired control of NF-kB activation and consequent continuous stimulation of B cells, enhance the risk of lymphoma. The first observation that specific pSS clinical features, such as parotid gland enlargement and lymphadenopathy, increase the risk of developing lymphoma dates back to the late 1970s [49] Several studies confirmed this finding and highlighted associations with other clinical and serological features [19,20,21]. The observation of high-BLyS levels in every pSS patient subgroup independently of the presence of a prelymphomatous condition or a fully blown lymphoma further supports the role of this molecule in every phase of pSS pathogenesis, including lymphomagenesis to induce and maintain B-cell survival. The demonstration of monoclonality using molecular biology testing allows for the differentiation of
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