原发性干燥综合征ppt课件.pptVIP

* * METHODS: Thirty-eight patients with ILD evaluated at our center underwent an minor salivary gland biopsy (MSGB) to confirm a diagnosis of pSS. We defined a positive MSGB finding as a lymphocyte focus score of 1. RESULTS: Thirteen patients (34%) had positive MSGB findings. Of these, 10 patients (77%) had sicca symptoms. Four patients (31%) were negative for both ANA and RF, and three patients (23%) were negative for ANA, RF, SS-A, and SS-B autoantibodies. CONCLUSIONS: Confirming a diagnosis of pSS-related ILD by performing MSGB allows for a more precise CTD classification. This study provides evidence that CTD may exist subclinically, and longitudinal studies are needed to determine whether identifying occult CTD impacts on management, longitudinal changes in lung function, or survival. * Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sj?grens syndrome. RESULTS: At diagnosis, 25% of pSS patients had GC-like structures in their salivary glands. Seven of the 175 patients studied (14% GC+ and 0.8% GC-) developed NHL during 1855 patient-years at risk, with a median onset of 7 years following the initial diagnostic salivary gland biopsy. Six of the seven patients had GC-like structures at diagnosis; the remaining patient was GC negative at the time of diagnosis (p=0.001). CONCLUSIONS: The detection of GC-like structures by light microscopy in pSS diagnostic salivary biopsies is proposed as a highly predictive and easy-to-obtain marker for NHL development. This allows for risk stratification of patients and the possibility to initiate preventive B-cell-directed therapy. * * * * * * * * 唾液腺、泪腺等腺体的分泌是受神经系统支配的，比如，我们吃饭时需要分泌腺体，大脑等就发出指令，指令通过副交感神传导，传到神经末梢时释放一种叫“乙酰胆碱”的东西，与腺体细胞表面的乙酰胆碱能受体（M受体）结合，从而促进腺体的分泌。M受体在我们人体的很多细胞上都有分布，它又可分为几种亚型，其中M3 受体主要与腺体的分泌有关，研究表面它主要存在于腺泡细胞膜上，部分存在于肌上皮细胞上，研究认为唾液腺的分泌可能是胆碱能神经的兴奋激活了腺泡细胞膜上的m3 受体，m3 受体激活后引起唾液分泌，同时可激活肌上皮细胞，引起肌上皮细胞收缩，协助腺泡细胞排出分泌物。 ???? 匹罗卡品（p ilocarp ine，