Abstract

Systemic autoimmune diseases (SADs) are a group of connective tissue diseases with diverse, yet overlapping, symptoms and autoantibody development. The etiology behind SADs is not fully elucidated, but a number of genetic and environmental factors are known to influence the incidence of SADs. Recent findings link dysregulation of Epstein-Barr virus (EBV) with SAD development. EBV causes a persistent infection with a tight latency programme in memory B-cells, which enables evasion of the immune defence. A number of immune escape mechanisms and immune-modulating proteins have been described for EBV. These immune modulating functions make EBV a good candidate for initiation of autoimmune diseases and exacerbation of disease progression. This review focuses on systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren's syndrome (SS) and sum up the existing data linking EBV with these diseases including elevated titres of EBV antibodies, reduced T-cell defence against EBV, and elevated EBV viral load. Together, these data suggest that uncontrolled EBV infection can develop diverse autoreactivities in genetic susceptible individuals with different manifestations depending on the genetic background and the site of reactivation.

Highlights

  • Systemic autoimmune diseases (SADs), called rheumatic connective tissue diseases, include rheumatoid arthritis (RA), Sjogren’s syndrome (SS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), systemic scleroderma (SSc), and dermatomyositis/polymyositis (DM/PM)

  • This review focuses on systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjogren’s syndrome (SS) and sum up the existing data linking Epstein-Barr virus (EBV) with these diseases including elevated titres of EBV antibodies, reduced T-cell defence against EBV, and elevated EBV viral load

  • These data suggest that uncontrolled EBV infection can develop diverse autoreactivities in genetic susceptible individuals with different manifestations depending on the genetic background and the site of reactivation

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Summary

Introduction

Systemic autoimmune diseases (SADs), called rheumatic connective tissue diseases, include rheumatoid arthritis (RA), Sjogren’s syndrome (SS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), systemic scleroderma (SSc), and dermatomyositis/polymyositis (DM/PM). EBV-specific cytotoxic T-cell reactivity has been observed to be reduced in SLE patients resulting in poor control of the EBV infection.

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