1,721,048 research outputs found
Might IgA be a biomarker of disease activity in Takayasu arteritis
Full text linkTakayasu arteritis is a systemic vasculitis of the large vessels and mainly affects Japanese and Southeast Asian women in the second and third decades of life. Inflammatory infiltrate affects the full thickness of the vessel wall, inducing progressive lumen stenosis and occlusion. The main biomarkers of disease activity are the ESR, CRP and serum levels of circulating cytokines. This case report describes the clinical history of a young woman with Takayasu disease with high serum levels of IgA at onset. IgA remained elevated with persistence of disease activity, and normalized only when the patient was treated with an anti-TNF agent (infliximab), which also induced a clinical response in the vasculitis. IgA levels, together with other inflammatory parameters, may be considered a biomarker of disease activity. Learning points: This case report highlights the need to increase the number of humoral markers used to assess disease course in Takayasu arteritis (TA).IgA may be considered a biomarker of TA disease activity.Serum IgA levels may be helpful to identify TA patients not responding to traditional therapy
The manufacture and usage of immunoglobulins for therapeutic use – The Dark Side of the Moon
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The Marginal Zone B Cells Clonally Expanded in Hepatitis C-Related Mixed Cryoglobulinemia Display the Functional and Molecular Signatures of Anergy Induced by Continual B Cell Receptor Signaling
No full textReplicative senescence prevents the differentiation of B-cells to antibody producine cells in common variable immunodeficiency
No full textHepatitis C virus-associated B-cell lymphomas: The importance of the new direct antiviral agent therapy
No full textHepatitis C virus (HCV) is a hepatotropic and lymphotropic virus, responsible for both chronic hepatitis and extra-hepatic manifestations. Multiple epidemiologic, clinical, biological, and molecular studies have suggested that HCV plays a causal role also in the development of several lymphoproliferative disorders, either benign, such as mixed cryoglobulinemia, or malignant, such as B-cell non-Hodgkin lymphomas (NHL). Chronic viral antigenic stimulation of B-lymphocytes plays a fundamental basic role from the on -set of lymphoma to its final steps. In the past, several studies demonstrated that the association of pe-gylated interferon plus ribavirin was able to eradicate HCV, with subsequent regression of indolent B-cell low-grade NHL. Other studies have demonstrated that direct antiviral agents (DAAs) therapy have some efficacy in HCV-associated NHL, particularly in patients with low-grade NHL or marginal zone-lymphoma, but these results need to be confirmed in larger studies with longer follow-up. The response rate of an-tiviral therapy seems favorable also in high grade NHL when DAAs therapy is administered in combina-tion with chemotherapy and therefore antiviral therapy should be considered as a first-line approach in HCV-related NHL.(c) 2022 Published by Elsevier Inc
A simultaneous occurrence of Tolosa-Hunt syndrome and fibrillary glomerulonephritis: a case report
No full textFibrillary glomerulonephritis (FibGN) is a rare cause of progressive renal dysfunction, often leading to dialysis within a few years. A 60-year-old woman presented with a 2 month history of right-sided retro-orbital pain and recent diplopia. Laboratory testing revealed an altered renal function with increased serum creatinine and mild proteinuria. MRI of the brain revealed the presence of a soft tissue mass on the right cavernous sinus compatible with the diagnosis of Tolosa-Hunt syndrome (THS). Renal biopsy showed a pattern compatible with fibrillary glomerulonephritis. For this reason steroid therapy was initiated at a dose of 1 mg/kg/day and adjusted according to the clinical course. Neurological symptoms regressed shortly after the beginning of therapy and renal function and proteinuria remained stable for the 3 years following the withdrawal of steroid therapy. Percutaneous renal biopsy was again performed and confirmed the previous diagnosis of FibGN in association with other glomerular-lesion-like mesangial widening, thickening of capillary walls and severe arterio-arteriolosclerosis. This case report describes what is believed to first report of the association of FibGN and THS, which both responded to steroid therapy
The CD21low B Cells Expanded in Common Variable Immunodeficiency do not Differentiate to Antibody Producing Cells After Stimulation of Toll-like Receptor 9 Because of a Reduced Proliferative Potential
No full textRelapsing cellulitis and bacteriemia due to a long lasting (10 years) Campylobacter coli infection in a XLA patient
No full textClonal expansion and functional exhaustion of monoclonal marginal zone B cells in mixed cryoglobulinemia: The yin and yang of HCV-driven lymphoproliferation and autoimmunity
No full textMonoclonal marginal zone (MZ) B cells expressing a VH1-69-encoded idiotype accumulate in HCV-associated mixed cryoglobulinemia (MC). These cells recognize the E2 protein of HCV and their massive clonal expansion reflects the propensity of MZ B cells to proliferate robustly upon antigenic stimulation by microorganisms, a property that makes them prone to neoplastic transformation. VH1-69+ B cells of MC patients are phenotypically heterogeneous and resemble either mature MZ B cells (IgM+CD27+CD21high) or the unusual CD21low B cells that accumulate in other immunological disorders such as common variable immunodeficiency (CVID) or HIV infection. The CD21low VH1-69+ B cells of MC patients, like those of CVID and HIV patients, are anergic to BCR and TLR9 stimulation and display deregulation of several anergy-related genes; proliferative anergy is also observed in CD21high MZ-like VH1-69+ B cells, that over-express the antiproliferative transcriptional repressor Stra13. Upon evolution to splenic marginal zone lymphoma, MZ-like VH1-69+ B cells down-regulate Stra13 and partially recover their capacity to proliferate in response to TLR9 ligation. Like yin and yang, robust clonal expansion and early proliferative anergy may be viewed as the opposite forces balancing the responses of human MZ B cells to chronic microbial stimuli. Disruption of this balance facilitates autoimmunity and lymphoproliferation. © 2012 Elsevier B.V
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