Search results for "Sjogren's Syndrome."
showing 10 items of 54 documents
A phase 2 randomized, double-blind, placebo-controlled, proof-of-concept study of oral seletalisib in primary Sjögren’s syndrome
2020
Abstract Objectives This phase 2 proof-of-concept study (NCT02610543) assessed efficacy, safety and effects on salivary gland inflammation of seletalisib, a potent and selective PI3Kδ inhibitor, in patients with moderate-to-severe primary Sjögren’s syndrome (PSS). Methods Adults with PSS were randomized 1:1 to seletalisib 45 mg/day or placebo, in addition to current PSS therapy. Primary end points were safety and tolerability and change from baseline in EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI) score at week 12. Secondary end points included change from baseline at week 12 in EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) score and histological features in salivary …
Is minor salivary gland biopsy more than a diagnostic tool in primary Sjorgren's syndrome? Association between clinical, histopathological, and molec…
2014
Objectives: Several histological scoring systems, including the focus score, performed in minor salivary glands (MSGs) by hematoxylin-eosin (H&E) staining, have been employed in clinical practice to assess the inflammatory infiltrate and provide the diagnosis of primary Sjorgren's syndrome (pSS). Aims of this study were to integrate different scoring systems and identify potential differences in the molecular profile of lymphoid cytokines related to germinal center (GC) formation and clinical subsets in pSS. Methods: Overall, 104 pSS patients and 40 subjects with sicca non-pSS were retrospectively evaluated. MSG biopsies were evaluated by H&E and immunofluorescence to assess histological pa…
A case of sjögren's syndrome with severe anemia due to myelitis
1986
An unusual case of Sjögren's syndrome presenting with severe anemia as the predominant clinical feature is described. Histological examination of a bone marrow biopsy specimen demonstrated that the patient's anemia was caused by myelitis and vasculitis of the small intraosseous vessels. Our report might stimulate a more thorough investigation of bone marrow in patients with connective tissue diseases and anemia.
Interleukin-36α axis is modulated in patients with primary Sjögren's syndrome.
2015
Summary The aim of this study was to investigate the expression of the interleukin (IL)-36 axis in patients with primary Sjögren's syndrome (pSS). Blood and minor labial salivary glands (MSG) biopsies were obtained from 35 pSS and 20 non-Sjögren's syndrome patients (nSS) patients. Serum IL-36α was assayed by enzyme-linked immunosorbent assay (ELISA). IL-36α, IL-36R, IL-36RA, IL-38, IL-22, IL-17, IL-23p19 and expression in MSGs was assessed by reverse transcription–polymerase chain reaction (RT–PCR), and tissue IL-36α and IL-38 expression was also investigated by immunohistochemistry (IHC). αβ and γδ T cells and CD68+ cells isolated from MSGs were also studied by flow cytometry and confocal …
Interleukin (IL)-22 receptor 1 is over-expressed in primary Sjogren's syndrome and Sjögren-associated non-Hodgkin lymphomas and is regulated by IL-18.
2015
Summary The aim of this study was to elucidate more clearly the role of interleukin (IL)-18 in modulating the IL-22 pathway in primary Sjögren's syndrome (pSS) patients and in pSS-associated lymphomas. Minor salivary glands (MSGs) from patients with pSS and non-specific chronic sialoadenitis (nSCS), parotid glands biopsies from non-Hodgkin lymphomas (NHL) developed in pSS patients, were evaluated for IL-18, IL-22, IL-22 receptor 1 (IL-22R1), IL-22 binding protein (IL-22BP) and signal transducer and activator of transcription-3 (STAT-3) expression. MSGs IL-22R1-expressing cells were characterized by confocal microscopy and flow cytometry in pSS, nSCS and healthy controls. The effect of recom…
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…
Rituximab in primary Sjögren's syndrome: a ten-year journey
2014
Primary Sjögren's syndrome (pSS) is an autoimmune disorder affecting exocrine glands and characterized in most cases by a rather mild clinical picture. However, a subgroup of pSS patients experience systemic extraglandular involvement leading to a worsening of disease prognosis. Current therapeutic options for the treatment of pSS are mainly empirical, often translated by other autoimmune diseases, and recent systematic reviews have highlighted the lack of evidence-based recommendations for most of the drugs commonly employed in the spectrum of extraglandular involvement. Because of the well-established role of B-lymphocytes in the pathogenesis of pSS, a B-cell targeting therapy may repres…
Re: ?Splenic marginal zone B-cell lymphoma associated with primary Sj�gren?s syndrome?
2003
We describe a 67-year-old woman with primary Sjögren's syndrome who developed a splenic marginal zone B-cell lymphoma, and complained of a dry mouth and Raynaud's phenomenon. She had splenomegaly, swollen lymph nodes and monoclonal IgM-kappa cryoglobulin. After splenectomy, Raynaud's phenomenon had improved with the decrease of cryoglobulin. This is the first case of primary Sjögren's syndrome with splenic marginal zone B-cell lymphoma to be reported.
Compromised central tolerance of ICA69 induces multiple organ autoimmunity
2014
For reasons not fully understood, patients with an organ-specific autoimmune disease have increased risks of developing autoimmune responses against other organs/tissues. We identified ICA69, a known β-cell autoantigen in Type 1 diabetes, as a potential common target in multi-organ autoimmunity. NOD mice immunized with ICA69 polypeptides exhibited exacerbated inflammation not only in the islets, but also in the salivary glands. To further investigate ICA69 autoimmunity, two genetically modified mouse lines were generated to modulate thymic ICA69 expression: the heterozygous ICA69(del/wt) line and the thymic medullary epithelial cell-specific deletion Aire-ΔICA69 line. Suboptimal central neg…
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…