Search results for "sjögren syndrome"
showing 10 items of 11 documents
Evaluation of labial microvessels in Sjogren syndrome: A videocapillaroscopic study
2008
Summary Sjogren's syndrome (SS) is a chronic autoimmune rheumatic disease characterized by a progressive lymphocytic infiltration of exocrine glands, especially salivary and lachrymal ones, leading to xerostomia, parotid gland enlargement, and xerophtalmia. SS may occur alone (primary) or in association with almost any of the autoimmune rheumatic diseases (secondary), the most frequent being rheumatoid arthritis. The aim of this study is to describe the capillaroscopic pattern of the labial mucosa in patients with SS. Methods A total of 20 patients affected by SS and 20 healthy controls were examined. The patients with conditions that compromise microcirculation, such as diabetes, hypertens…
Clinical and laboratorial profile and histological features on minor salivary glands from patients under investigation for Sjogren´s syndrome
2013
Diagnosis of Sjögren's syndrome (SS) is complex and the usefulness of labial minor salivary glands biopsy in this process remains controversial. Objectives: to evaluate the clinical and laboratorial profile and histological features on labial minor salivary glands from patients under investigation of SS. Study D esign: clinical charts from 38 patients under suspicion of SS and submitted to labial minor salivary glands biopsies were reviewed. Clinical and laboratorial data were retrieved from the clinical files and the HE-stained histological slides were reviewed under light microscopy. Results: mean age of the patients was 56.5 years and 97% were females; histological analysis showed that 4…
SIL1 mutations and clinical spectrum in patients with Marinesco-Sjogren syndrome.
2013
Marinesco-Sjogren syndrome is a rare autosomal recessive multisystem disorder featuring cerebellar ataxia, early-onset cataracts, chronic myopathy, variable intellectual disability and delayed motor development. More recently, mutations in the SIL1 gene, which encodes an endoplasmic reticulum resident co-chaperone, were identified as the main cause of Marinesco-Sjogren syndrome. Here we describe the results of SIL1 mutation analysis in 62 patients presenting with early-onset ataxia, cataracts and myopathy or combinations of at least two of these. We obtained a mutation detection rate of 60% (15/25) among patients with the characteristic Marinesco-Sjogren syndrome triad (ataxia, cataracts, m…
Salivary gland cancer in Southern Brazil: a prognostic study of 107 cases.
2020
Background Salivary gland cancers (SGC) represent an uncommon group of heterogeneous tumors. We performed a retrospective survey of SGC diagnosed in a reference center for treatment of malignant tumors from the south of Brazil aiming to determine the prognostic value of demographic, clinic and pathologic features. Material and Methods Cases diagnosed as SGC between 2006 and 2016 were retrospectively collected. Medical records were examined to extract demographic, clinic, pathologic and follow-up information. Results One-hundred and seven cases of SGC were identified. The most common SGC were mucoepidermoid carcinoma (MEC) (n = 39) followed by adenoid cystic carcinoma (AdCC) (n = 29). Among …
CD4 T lymphocyte autophagy is upregulated in the salivary glands of primary Sjögren’s syndrome patients and correlates with focus score and disease a…
2017
Background Primary Sjögren’s syndrome (pSS) is a common chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and peripheral lymphocyte perturbation. In the current study, we aimed to investigate the possible pathogenic implication of autophagy in T lymphocytes in patients with pSS. Methods Thirty consecutive pSS patients were recruited together with 20 patients affected by sicca syndrome and/or chronic sialoadenitis and 30 healthy controls. Disease activity and damage were evaluated according to SS disease activity index, EULAR SS disease activity index, and SS disease damage index. T lymphocytes were analyzed for the expression of autophagy-specific marke…
Stimulated whole salivary flow rate: The most appropriate technique for assessing salivary flow in Sjogren syndrome
2020
Background We sought to determine the most appropriate method for measuring salivary flow to aid the diagnosis of Sjögren's syndrome (SS). Specifically, we compared the unstimulated whole salivary flow rate (UWSFR) with the stimulated whole salivary flow rate (SWSFR). Material and Methods This case-control study comprised one group of 103 patients with SS and a control group of 50 healthy people. We measured the UWSFR and SWSFR in both groups according to the guidelines established by Navacet (1993). Results The UWSFR and SWSFR were significantly lower in the patient group compared with the controls (p < 0.01). Among the participants in the patient group, we found a decreased UWSFR in 84 in…
Marinesco-Sjögren syndrome caused by a new SIL1 frameshift mutation
2015
no abstract available
Guidelines for biomarkers in autoimmune rheumatic diseases - evidence based analysis
2018
Autoimmune rheumatic diseases are characterised by an abnormal immune system response, complement activation, cytokines dysregulation and inflammation. In last years, despite many progresses in managing these patients, it has been shown that clinical remission is reached in less than 50% of patients and a personalised and tailored therapeutic approach is still lacking resulting in a significant gap between guidelines and real-world practice. In this context, the need for biomarkers facilitating early diagnosis and profiling those individuals at the highest risk for a poor outcome has become of crucial interest. A biomarker generally refers to a measured characteristic which may be used as a…
Cardiovascular disease in primary sjögren’s syndrome
2018
Background A close relationship between rheumatic diseases and cardiovascular disease (CVD) has been reported, accounting for the higher mortality and morbidity observed in these patients. In the last years, it has been clearly reported that patients affected by primary Sjogren's syndrome (pSS) experienced an increased risk of CVD. Objective This review aimed at investigating CVD, traditional cardiovascular (CV) risk factors and possible targeted therapeutic strategies in pSS patients. Method Available literature concerning CV risk factors in pSS patients has been selected and discussed. Conclusion Disease-related characteristics and traditional CV risk factors contribute to observed athero…
Assessing the determination of salivary electrolytes and anti-Ro and anti-La antibodies for the diagnosis of Sjogren s syndrome (SS)
2009
The aim of this study was to assess changes in salivary electrolyte flow and composition and the presence of anti-Ro/SSA and anti-La/SSB serum and saliva antibodies and their implications for the non-invasive diagnosis of SS. Study design: 73 patients were studied, divided into the following experimental groups: primary Sjögren syndrome (SSp) (n=15), secondary SS (SSs) (n=17), dry mouth, dry eye without Sjögren?s syndrome (BO) (n=20) and healthy controls (C) (n=21). We conducted a baseline assessment of salivary flow and saliva sampling for the measurement of sodium, chlorine, potassium, calcium and phosphate electrolytes, and the determination of antiRo/SSA and La/SSB antibodies; a serum s…