Moutsopoulos H M, Manoussakis M N
Department of Internal Medicine, School of Medicine, University of Ioannina, Greece.
Autoimmunity. 1989;5(1-2):17-24. doi: 10.3109/08916938909029139.
Sjogren's syndrome (Ss) is an ideal model to study the pathogenesis of both autoimmunity and malignancy. It occurs as an organ specific autoimmune disease, alone or in association with almost every other autoimmune disorder, as a systemic disorder, and finally it can evolve to B-cell-lymphoid malignancy. The most consistent finding in the syndrome, the B-cell-hyperreactivity, follows the same steps of evolution. It starts as polyclonal, but not random, since the autoantibody profile correlates with the disease subgroups and the systemic manifestations and it seems to be controlled by the MHC gene composition. Further, in the systemic form of the disease it presents as a poly-oligo-mono-clonal process and ends up to monoclonal (IgMk) B-lymphoid malignancy. Studies on the T-immunoregulatory subsets and function can not explain this B-cell hyperreactivity. The initial trigger is unknown. Estrogens, known as immunoenhancers possibly promote the B-cell hyperreactivity and certain genes controlling HLA class-II MHC molecules may represent susceptibility factors for the development of the disease. The discovery of lymphokines and particularly the B-cell growth and differentiation factors as well as the rapid development of the retro-virology field may give answers pertinent to the pathogenesis of Ss and to B-cell lymphoid malignancy.
干燥综合征（Ss）是研究自身免疫和恶性肿瘤发病机制的理想模型。它作为一种器官特异性自身免疫性疾病单独出现，或与几乎所有其他自身免疫性疾病相关联，作为一种全身性疾病出现，最终可演变为B细胞淋巴瘤。该综合征最一致的发现，即B细胞高反应性，遵循相同的演变步骤。它始于多克隆，但并非随机，因为自身抗体谱与疾病亚组和全身表现相关，且似乎受MHC基因组成控制。此外，在疾病的全身性形式中，它表现为多克隆-寡克隆-单克隆过程，最终发展为单克隆（IgMk）B细胞淋巴瘤。对T免疫调节亚群及其功能的研究无法解释这种B细胞高反应性。最初的触发因素尚不清楚。雌激素作为免疫增强剂，可能促进B细胞高反应性，而某些控制HLA II类MHC分子的基因可能是该疾病发展的易感因素。淋巴因子的发现，特别是B细胞生长和分化因子的发现，以及逆转录病毒学领域的快速发展，可能为干燥综合征的发病机制和B细胞淋巴瘤提供相关答案。
