Common and rare forms of vasculitis associated with Sjögren's syndrome

Purpose of review Although uncommon, systemic vasculitis is one of the most severe extraglandular manifestations of primary Sjögren's syndrome (pSS) accounting for the increased morbidity and mortality of the disease. This review aims to describe major previous and recent reports regarding the clinical presentation, prognosis and treatment of systemic vasculitis associated with pSS. Recent findings Both older and recent pSS cohort studies performed over the past several and recent years, have clearly shown that cryoglobulinaemic vasculitis is the most frequent type of systemic vasculitis accompanying pSS. Antineutrophil cytoplasmic antibody-associated, large and medium vessel vasculitis are described only in sporadic cases. In addition to the overt clinical manifestations of cryoglobulinaemic vasculitis, type II cryoglobulinaemia, glomerulonephritis and purpura have been correlated with increased risk for B-cell non-Hodgkin lymphoma (NHL) in pSS. Summary pSS is characterized by autoreactive B and T-cell infiltrates around the epithelial structures of the affected organs, as well as, B-cell hyperreactivity. The latter, is attested by the increased production of autoantibodies, directed against many different organ and nonorgan self-antigens. Vasculitis is a significant and potentially life-threatening complication of the disease depending on the size, localization, histologic type and the pathogenetic mechanisms of the inflammatory process. The potentially irrever...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: VASCULITIS SYNDROMES: Edited by Hasan Yazici and Yusuf Yazici Source Type: research

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Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
In conclusion, we show a novel mouse model of coronary stenosis induced by LCWE that is characterized by coronary stenosis with severe coronary vasculitis and elastin degradation. In addition, VSMC proliferation plays an important role in the formation of coronary stenosis. This model is an appropriate model of KD coronary stenosis. PMID: 31932543 [PubMed - as supplied by publisher]
Source: Experimental Animals - Category: Research Tags: Exp Anim Source Type: research
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Source: Cancer Cell International - Category: Cancer & Oncology Authors: Tags: Correction Source Type: research
To investigate the clinical implications of SF3B1 and ring sideroblasts in myelodysplastic syndrome-refractory cytopenia with multilineage dysplasia (MDS-RCMD), 238 consecutive MDS-RCMD patients were included in this study. Our results showed that SF3B1 mutation in MDS- RCMD patients identifies a homogenous phenotype characterized by homogeneous hematologic features including a high prevalence of erythroid dysplasia and a high proportion of ring sideroblasts, prolonged survival and reduced leukemic transformation.
Source: Clinical Lymphoma, Myeloma and Leukemia - Category: Hematology Authors: Tags: Original Study Source Type: research
The anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are a group of life-threatening multi-system diseases characterized by necrotising inflammation of small blood vessels and crescentic glomerulonephritis. ANCA are thought to play a direct pathogenic role. Previous studies have shown that spleen tyrosine kinase (SYK) is phosphorylated during ANCA-induced neutrophil activation in vitro. However, the role of SKY in vivo is unknown. Here, we studied its role in the pathogenesis of experimental autoimmune vasculitis, a pre-clinical model of myeloperoxidase-ANCA-induced pauci-immune systemic vasculitis i...
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Basic Research Source Type: research
AbstractObinutuzumab is a glycoengineered tumor-targeting anti-CD20 mAb with a modified crystallizable fragment (Fc) domain designed to increase the affinity for the Fc γRIIIA/CD16 receptor, which was recently approved for clinical use in CLL and follicular lymphoma. Here we extend our previous observation that, in human NK cells, the sustained CD16 ligation by obinutuzumab-opsonized targets leads to a markedly enhanced IFN-γ production upon a subsequent cytokine re-stimulation. The increased IFN-γ competence in response to IL-2 or IL-15 is attributable to post-transcriptional regulation, as it does not c...
Source: Cancer Immunology, Immunotherapy - Category: Cancer & Oncology Source Type: research
Source: Neurological Sciences - Category: Neurology Source Type: research
Condition:   NK/T Cell Lymphoma Interventions:   Drug: IV Nivolumab;   Drug: IV Gemcitabine;   Drug: IV Cisplatin;   Drug: IV/PO Dexamethasone;   Drug: IV L-asparaginase Sponsors:   National Cancer Centre, Singapore;   Bristol-Myers Squibb Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
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Source: Journal of Hepatology - Category: Gastroenterology Authors: Tags: Review Source Type: research
Abstract Cryoglobulinemic vasculitis (CV) is a small-to-medium-vessel vasculitis that appears in 10–15 % of patients chronically infected with hepatitis C virus (HCV). The classic symptom triad of CV, purpura/asthenia/arthralgia, is accompanied by clinical features that include glomerulonephritis, neuropathy, interstitial pneumonitis, and cardiomyopathy, ranging in their severity from mild to life threatening. The risk of developing non-Hodgkin lymphoma is also higher. The cumulative 10-year survival rate of CV patients is significantly lower than in the age- and sex-matched general population, with dea...
Source: Clinical and Experimental Medicine - Category: Research Source Type: research
More News: Glomerulonephritis | Hodgkin's Disease | Lymphoma | Non-Hodgkin's Lymphoma | Rheumatology | Study | Vasculitis