Title:Possible Mechanisms of Lymphoma Development in Sjogren’s Syndrome
Volume: 9
Issue: 1
Author(s): Lingli Dong, Yu Chen, Yasufumi Masaki, Toshiro Okazaki and Hisanori Umehara
Affiliation:
Keywords:
Incidence of lymphoma development, lymphoma, lymphoproliferative disease, Sjogren’s syndrome, therapeutic
progression.
Abstract: Primary Sjogren’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.
