Abstract
A clinical picture of dry eye and dry mouth with the histological counterpart of focal lymphocytic sialoadenitis, usually detected in minor salivary glands, is considered the hallmark of Sjögren's syndrome. The association of sicca complaints and focal sialoadenitis can be also found in a number of other diseases, including some systemic viral infections. Among these conditions, chronic hepatitis C virus infection, associated with mixed cryoglobulinaemia and extra-hepatic manifestations, and HIV infection, particularly in the phase of diffuse interstitial lymphocytic infiltration, may mimic the clinical and histological aspects of Sjögren's syndrome. However, each disorder is characterised by specific, disease-related immunopathological aspects. Besides sicca complaints, the various disorders may also share a number of systemic extra-glandular features and the possible development of mucosa-associated lymphoid tissue lymphomas. This latter event represents in all of these diseases the final result of an antigen-driven chronic stimulation of B lymphocytes.
Highlights
Sjögren’s syndrome (SS) is a chronic systemic autoimmune disease mainly affecting the exocrine glands
SS has received the attribute primary when it appears alone, or secondary when it is associated with another well-defined systemic autoimmune disease, such as systemic lupus, rheumatoid arthritis or systemic sclerosis [4]
This study clearly indicates a direct role for these viral proteins in the pathogenesis of hepatitis C virus (HCV)-related sialoadenitis
Summary
Sjögren’s syndrome (SS) is a chronic systemic autoimmune disease mainly affecting the exocrine glands. This is undoubtedly due to the greater attention devoted to the predominance of liver involvement in HCV infection, and to the fact that features different from sicca complaints are more clinically significant in HCV infection with extra-hepatic manifestations. DILS differs from pSS by the fact that the occurrence of extra-glandular involvement is more frequent, whilst the presence of autoantibodies and rheumatoid factor is observed in a lower number of patients In addition, the two conditions differ in the nature of infiltrating lymphocytes (CD4+ T cells in pSS, CD8+ T cells in DILS) and in the association with different HLA haplotypes [58,59,60]. This observation again suggests that infection-related lymphomas derived from MZ B cells may share common antigen-driven mechanisms [50]
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