Witte T
Klinik für Immunologie und Rheumatologie, Carl-Neuberg-Str. 1, 30625 Hannover.
Z Rheumatol. 2010 Feb;69(1):50-6. doi: 10.1007/s00393-009-0519-2.
Sjögren's syndrome is a common autoimmune disorder. Several genetic risk factors such as STAT-4, ILT6 and the haplotype HLA-B8/DR3 have been identified. In addition, there are environmental risk factors, possibly chronic viral infections. In the pathophysiology of Sjögren's syndrome T and B cells infiltrate the salivary and lacrimal glands. As a consequence of the destruction of glandular cells by cytotoxic T cells, production of cytokines and autoantibodies inhibiting glandular function, the production of saliva and tears is decreased. The feeling of dry eyes and mouth is frequently not noticed by the patients. Therefore, Sjögren's syndrome should also be considered when extraglandular manifestations such as vasculitis, polyneuropathy or arthritis occur, even when the patients do not complain of dry eyes and mouth. Establishing the diagnosis of Sjögren's syndrome requires verification of reduced glandular function, for example using Schirmer's test and the Saxon test. The confirmation of Sjögren's syndrome as a cause of sicca syndrome is subsequently performed by the detection of autoantibodies against Ro (SS-A) and La (SS-B) and/or by a salivary gland biopsy.
干燥综合征是一种常见的自身免疫性疾病。已确定了几种遗传风险因素,如信号转导和转录激活因子4(STAT-4)、免疫球蛋白样转录物6(ILT6)以及单倍型人类白细胞抗原B8/DR3(HLA-B8/DR3)。此外,还存在环境风险因素,可能是慢性病毒感染。在干燥综合征的病理生理学中,T细胞和B细胞浸润唾液腺和泪腺。由于细胞毒性T细胞破坏腺细胞、细胞因子和抑制腺功能的自身抗体产生,唾液和泪液的分泌减少。患者常常没有注意到眼睛和口腔干燥的感觉。因此,即使患者没有诉说眼睛和口腔干燥,当出现血管炎、多发性神经病或关节炎等腺外表现时,也应考虑干燥综合征。确立干燥综合征的诊断需要验证腺功能减退,例如使用施密特试验(Schirmer试验)和萨克森试验(Saxon试验)。随后通过检测抗Ro(SS-A)和La(SS-B)自身抗体和/或进行唾液腺活检来确认干燥综合征是干燥综合征的病因。