Insight into pathogenesis of Sjögren's syndrome: Dissection on autoimmune infiltrates and epithelial cells.
Sjögren's syndrome (SS) is a chronic autoimmune disease with broad clinical spectrum, extending from benign exocrinopathy to severe systemic disease and lymphoma development. The glandular and extraglandular dysfunction of SS is associated with lymphocytic infiltrates that invade the epithelial structures of affected organs. The in-depth study of autoimmune lesions in the minor salivary glands (MSG), which are the major target-organ of SS responses, revealed that the lymphocytic infiltrates vary in severity and composition among SS-patients, are full-blown at diagnosis and remain unchanged thereafter. Although the pathogenetic pathways underlying SS have not yet elucidated, it is well-established that glandular epithelial cells are central regulators of local autoimmune responses. Moreover, chronic inflammation affects epithelial function and phenotype, which strengthens or weakens their immunoregulatory/secretory function, leading to deterioration of autoimmune phenomena. Herein, the current findings regarding the autoimmune lesions, the role of epithelial cells and their interaction with infiltrating lymphocytic cells are discussed.
Department of Pathophysiology & Academic Joint Rheumatology Program, School of Medicine, National University of Athens, Greece; Johns Hopkins University, Department of Medicine, Rheumatology Division, Baltimore, USA.
Andreas V
AV
Goules
Department of Pathophysiology & Academic Joint Rheumatology Program, School of Medicine, National University of Athens, Greece.
Efstathia K
EK
Kapsogeorgou
Department of Pathophysiology & Academic Joint Rheumatology Program, School of Medicine, National University of Athens, Greece. Electronic address: agtzi@med.uoa.gr.
Athanasios G
AG
Tzioufas
D015551:Autoimmunity / Q000276:immunology*; D003713:Dendritic Cells / Q000276:immunology / Q000473:pathology; D004847:Epithelial Cells / Q000276:immunology*; D006801:Humans; D007249:Inflammation; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology; D007962:Leukocytes / Q000276:immunology* / Q000473:pathology; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology; D008264:Macrophages / Q000276:immunology / Q000473:pathology; D012470:Salivary Glands, Minor / Q000166:cytology / Q000276:immunology* / Q000473:pathology; D012859:Sjogren's Syndrome / Q000276:immunology* / Q000473:pathology
Autoimmune responses; Infiltrates; Pathogenesis; Salivary gland epithelial cells; Sjögren's syndrome