Possible Mechanisms of Lymphoma Development in Sjogren’s Syndrome

Abstract

Primary Sjögren’s syndrome (pSS) is a systemic as well as an organ-specific autoimmune disease characterized by lymphocytic infiltration of the glandular epithelial tissue. SS patients have been reported to be at highest risk of developing lymphoproliferative neoplasms, when compared with patients with other rheumatoid diseases. Factors such as cytokine stimulation, environmental factors, viral infection and genetic events as well as vitamin deficiency may contribute to the development of lymphoma. Over the past few decades, numerous efforts have been made to assess the relationship between lymphoma and SS. These include epidemiological surveys, molecular biologic assessments of clonality and well-linked register cohort studies evaluating the predictive value of clinical, laboratory and histological findings. Nevertheless, the mechanisms and factors predictive of lymphoma development in pSS patients remain to be defined. This review summarizes updated knowledge on the incidence of and risk factors for lymphoma development in pSS patients, as well as discussing the most recent findings on the development and treatment of lymphoma in pSS patients and the possible mechanism of lymphoma development.

Characteristics of germinal center-like structures in patients with Sjögren's syndrome

“…Compared to the normal population, patients with primary SS manifested aberrant B‐cell activity, exocrine gland infiltration, production of various antibodies and hyper‐immunoglobulin response, and were reported to have a 16‐ to 44‐fold higher risk of developing lymphoma . The high serum immunoglobulin levels and high risk of malignant transformation both seem to be associated with abnormal ectopic GC formation in autoimmune diseases …”

“…Compared to the normal population, patients with primary SS manifested aberrant B-cell activity, exocrine gland infiltration, production of various antibodies and hyper-immunoglobulin response, and were reported to have a 16-to 44-fold higher risk of developing lymphoma. 13,14 The high serum immunoglobulin levels and high risk of malignant transformation both seem to be associated with abnormal ectopic GC formation in autoimmune diseases. 15 GCs are specialized microenvironments within secondary lymphoid tissues in which B cells undergo extensive rounds of proliferation, somatic hypermutation and antigen affinity-driven selection, 15,16 especially in antibody-related autoimmune diseases such as SS, SLE and RA.…”

Primary Pulmonary Hodgkin Lymphoma Associated with Primary Sjögren's Syndrome

“…The relative risk of developing lymphoma was about 16-fold higher in SS patients than in the general population [4]. Most lymphomas associated with SS are extranodal marginal zone lymphomas, followed by diffuse large B-cell lymphomas [4].…”

“…Most lymphomas associated with SS are extranodal marginal zone lymphomas, followed by diffuse large B-cell lymphomas [4].…”

Dental perspective on Sjögren’s syndrome: literature review

“…In the case of SS the risk has been estimated to be 44 times that of the general population 73 . It has been reported that stimulation of cytokines, parotidomegaly 74 , environmental factors, viral infections 75 and vitamin deficiencies predispose to the development of these lesions 76 .…”