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1.
A 20 year old man with beta thalassaemia developed symptoms of the sicca syndrome. His serum contained rheumatoid factor and antinuclear antibodies. A biopsy specimen of labial salivary gland showed large accumulations of haemosiderin within the parenchymal cells of the acini. Although in this case the sicca syndrome could not be definitely distinguished from Sj?gren's syndrome, the patient's HLA type was not the one usually associated with Sj?gren's syndrome. Histological appearances suggested that the causative factor of the sicca syndrome was iron overload owing to an intensive blood transfusion regimen.  相似文献   

2.
The term Sj?gren's syndrome refers to keratoconjunctivitis sicca and xerostomia due to lymphocytic infiltrates of lachrymal and salivary glands. The current used criteria for diagnosis of primary Sj?gren's syndrome is the American-European consensus. Primary Sj?gren's syndrome is an autoimmune disorder characterized by lymphocytic infiltrates and destruction of the salivary and lachrymal glands and systemic production of autoantibodies to the ribonucleoprotein particles SS-A/Ro and SS-B/La. The infiltrating cells (T- and B-cells, dendritic cells) interfere with glandular function at several points: destruction of glandular elements by cell-mediated mechanisms; secretion of cytokines that activate pathways bearing the signature of type 1 and 2 interferons; production of autoantibodies that interfere with muscarinic receptors; and secretion of metalloproteinases (MMPs) that interfere with the interaction of the glandular cell with its extracellular matrix, which is necessary for efficient glandular function. As the process progresses, the mucosal surfaces become sites of chronic inflammation and the start of a vicious circle. Despite extensive study of the underlying cause of Sj?gren's syndrome, the pathogenesis remains obscure. In broad terms, pathogenesis is multifactorial; environmental factors are thought to trigger inflammation in individuals with a genetic predisposition to the disorder.  相似文献   

3.
为了探索早期诊断干燥综合征患者合并恶性淋巴瘤,作者采用B细胞恶性淋巴瘤基因探针检测了45例干燥综合征患者的泪腺组织及患眼附属器B恶性淋巴瘤组织。结果4例干燥综合征患者泪腺组织阴性,而5例恶性淋巴瘤患者的瘤组织扩增阳性。结果表明,一般的原发或继发性干燥综合征患者泪腺中的淋巴细胞中无恶性B淋巴瘤基因。  相似文献   

4.
Predictive value of SS-B precipitating antibodies in Sjo?gren's syndrome   总被引:1,自引:0,他引:1  
As part of a screening programme several patients were identified with antibodies to the nuclear antigen SS-B. Fifteen were examined and 11 found to have Sj?gren's syndrome, though this had not been suspected by most of the referring physicians. In contrast, among a group of 17 patients with overt Sj?gren's syndrome, most of whom also had rheumatoid arthritis, only one had antibodies to SS-B. Patients presenting with polyarthralgia found to be SS-B positive may be likely to develop Sj?gren's syndrome but unlikely to develop rheumatoid arthritis. The detection of SS-B antibodies may antedate clinical evidence of Sj?gren's syndrome by months or even years. These results emphasise the clinical heterogeneity of Sj?gren's syndrome.  相似文献   

5.
OBJECTIVE: To evaluate the incidence, severity, clinical manifestations and immunological features relevant to liver involvement in 135 cases of primary Sj?gren's syndrome. METHODS: One hundred and thirty-five patients with definite primary Sj?gren's syndrome were analyzed retrospectively for liver involvement by the abnormalities of the liver enzymes, bilirubin level and liver biopsied section. RESULTS: The liver involvement in 30 patients (22.2%) could be etiologically ascribed to Sj?gren's syndrome itself. The clinical spectrum and severity of this entity differed widely, 36.6% showed no relevant clinical symptoms, however jaundice was found in 46.7% of patients. Six patients showed pathological changes of chronic active hepatitis. 73.3% of all patients with liver involvement responded to steroid and immunosuppressive drugs, yet with a tendency to relapse (two cases). Liver cirrhosis was developed in five cases. The spectrum of serum autoantibodies in the patients with liver involvement showed no difference from those without liver involvement. Most of them were compatible with the serum profile of autoimmune hepatitis type-1. CONCLUSIONS: Liver involvement was complicated in 22.2% patients of primary Sj?gren's syndrome. Clinical manifestations were non-specific and the main pathological change was chronic active hepatitis. The differential diagnosis between Sj?gren's syndrome with liver involvement and type-1 autoimmune hepatitis could be only ascribed to other systemic clinical manifestations of Sj?gren's syndrome.  相似文献   

6.
张朋  赵莹  杨舒婷  李江 《海南医学》2016,(13):2171-2173
舍格伦综合征是一种具有独特特征且临床症状多样的慢性自身免疫性疾病。该病在临床上分为原发和继发两类,从局部外分泌腺病变到系统性免疫疾病、淋巴瘤等均可发生,严重影响患者生活质量。关于该病的病因和发病机制尚未完全明确,有许多问题有待进一步研究。  相似文献   

7.
Primary Sj?gren's syndrome is a chronic autoimmune disease that can lead to systemic manifestations. At present, immunomodulatory agents have not shown good ef?cacy, many patients in China seek Chinese medicine treatment. Chinese medicine can comprehensively improve the symptoms of patients through Chinese pattern diagnosis and individualized treatment. Fundamental researches are providing scienti?c bases for the therapeutic effect of Chinese medicine. Professional Chinese medicine treatment can be integrated into the conventional management of primary Sj?gren's syndrome.  相似文献   

8.
目的:了解干燥综合征患者外周血中CD5+B细胞的变化及其B淋巴细胞CD5在基因水平上的表达,探讨CD5+B细胞与干燥综合征的关系。方法:采用免疫荧光双标记技术和流式细胞仪检测了20例干燥综合征患者和10例健康对照者外周血中CD5+B细胞(CD5+CD19+)的百分率。应用流式细胞仪分选B淋巴细胞的基础上,运用Real-time RT-PCR方法检测7例干燥综合征患者外周血CD5 mRNA的表达水平。结果:干燥综合征患者外周血CD5+B(CD5+CD19+)细胞的百分率高于健康对照组(P<0.05)。86%的患者CD5 mRNA高于正常人水平。结论:干燥综合征患者外周血CD5+B细胞及CDS转录水平和干燥综合征的发病有一定的关联。  相似文献   

9.
近年来毒邪致病理论在干燥综合征的认识和治疗中得到越来越多的应用和研究。对干燥综合征毒邪致病学说的中医观点和现代学研究进行总结, 探讨了中医毒邪致病理论与现代医学干燥综合征发病机制的联系, 以期为干燥综合征的治疗与研究提供新思路、新方法。   相似文献   

10.
腮腺造影唇腺活检对Sjoegren综合征的诊断价值   总被引:2,自引:0,他引:2  
对100例Sjoegren综合征患者进行腮腺造影及唇腺活辁、组织病理学检查,87例患者显示腮腺末梢导管扩张性改变;90例显示局灶性淋巴细胞浸润,符合郑麟善氏Ⅰ级,59例符合Chisholm 4级,结果提示1疾病中两种腺体损害的一致性,也证实了捐害的不均衡性。本研究提示了一种联合观察腮腺造影和唇腺活检结果诊断涎腺损害的新方法。  相似文献   

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