ABSTRACT
Primary Sjögren syndrome (pSjS) is an autoimmune disease, in which a large number of lymphocytes infiltrate the exocrine glands and cause a glandular dysfunction. Its pathogenesis is related to the chronic inflammation of the exocrine glands caused by genetic factors, viral infection and cell-mediated mechanisms. Long-term inflammation leads to accelerated apoptosis of epithelial cells, disordered gland structure, increased expression of proinflammatory cytokines such as CXC chemokine family ligand (CXCL)12, CXCL13, B cell-activating factor, interleukin-6, interferon-gama and tumor necrosis factor-alfa in submandibular glands. Under the action of antigen-presenting cells such as dendritic cells, macrophages and other antigen-presenting cells induces lymphocytes (mainly B lymphocytes) to mature in secondary lymphoid organs and migrate to the submandibular glands to promote the formation of germinal centers and autoantibody synthesis. Meanwhile, innate lymphocytes, vascular endothelial cells and mucosa-associated constant T cells as important immune cells, also participated in the inflammatory response of the submandibular gland in pSS through different mechanisms. Various signaling pathways interact with each other and are intricately complex, causing lymphocytes to continuously activate and invade the submandibular glands. As the process progresses, the mucosal surfaces become sites of chronic inflammation and the vicious circle starts.
Keywords: Sjögren's syndrome; autoimmunity; immunological factors; pathophysiology; pathogenesis
Primary Sjögren syndrome (pSjS) is an autoimmune disease, in which a large number of lymphocytes infiltrate the exocrine glands and cause a glandular dysfunction. Its pathogenesis is related to the chronic inflammation of the exocrine glands caused by genetic factors, viral infection and cell-mediated mechanisms. Long-term inflammation leads to accelerated apoptosis of epithelial cells, disordered gland structure, increased expression of proinflammatory cytokines such as CXC chemokine family ligand (CXCL)12, CXCL13, B cell-activating factor, interleukin-6, interferon-gama and tumor necrosis factor-alfa in submandibular glands. Under the action of antigen-presenting cells such as dendritic cells, macrophages and other antigen-presenting cells induces lymphocytes (mainly B lymphocytes) to mature in secondary lymphoid organs and migrate to the submandibular glands to promote the formation of germinal centers and autoantibody synthesis. Meanwhile, innate lymphocytes, vascular endothelial cells and mucosa-associated constant T cells as important immune cells, also participated in the inflammatory response of the submandibular gland in pSS through different mechanisms. Various signaling pathways interact with each other and are intricately complex, causing lymphocytes to continuously activate and invade the submandibular glands. As the process progresses, the mucosal surfaces become sites of chronic inflammation and the vicious circle starts.
Keywords: Sjögren's syndrome; autoimmunity; immunological factors; pathophysiology; pathogenesis
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