Chronic Epstein-Barr virus infection: A potential junction between primary Sjögren’s syndrome and lymphoma

Primary Sjögren’s syndrome (pSS) is an autoimmune disorder that progresses slowly and has a low incidence and fatality rate, characterized by B cell hyperactivation and inflammation in exocrine glands, including the salivary gland (SG) and lacrimal gland. These abnormally-activated B cells produce autoantibodies targeting Sjögren’s syndrome-associated proteins named SSA and SSB and damage the epithelium of exocrine glands, leading to the dry eyes/ xerophthalmia and dry mouth/ xerostomia [1].

In addition, there are extra-glandular systemic manifestations, such as fatigue, cutaneous involvement, and cardiovascular abnormalities [2], [3]. The development of lymphoma is a significant complication of pSS progression, resulting in an increased mortality rate for pSS patients developing lymphoma during their diseases [4]. Patients who developed lymphoma after the diagnosis of pSS showed a lower survival rate than those who were diagnosed with haematological malignancy at least one year prior to the diagnosis of pSS [5].

Although the pathophysiology of pSS is unclear, it is presently assumed to be associated with environmental and genetic factors, resulting in abnormal immunology. Epstein-Barr virus (EBV) infection has been considered as a significant environmental risk factor due to its ability to cause epithelial damage and stimulate the innate and adaptive immune systems [6], [7]. Researchers detected EBV DNA by polymerase chain reaction and in situ hybridization, showing that the frequency of EBV-positive salivary epithelial cells is higher in pSS patients [8]. Recent research has provided crucial serological evidence linking EBV infection and pSS [9], [10]. Although latent infection does not compromise the health of the majority of patients, EBV-infected patients might be more susceptible to lymphoproliferative disorders and lymphoma in the latency or lytic infection phase [11].

This review aims to elaborate the involvement of EBV infection in the development of pSS and analyze whether EBV infection increases the risk of lymphoma in pSS patients.

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