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Vasculitis - Europe PMC

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Last Updated: 27 August 2022

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Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge.

Patients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed. Methods Because of persistent renal failure, six patients received immunosuppressive therapy before antibiotics due to misdiagnosis, and seven patients were given immunosuppressive therapy after antibiotics. Key factsInfective endocarditis-related RPGN is uncommon, and distinguishing it from idiopathic vasculitis is difficult, and diagnosis of infectious endocarditis is seldom, and antibiotic therapy alone is often helpful in patients with unusual kidney dysfunction.

Source link: https://europepmc.org/article/MED/35243934


Identification of differentially expressed genes and pathways in kidney of ANCA-associated vasculitis by integrated bioinformatics analysis.

A system of extremely rare autoimmune disorders with no known cause is found in the antineutrophil cytoplasmic antibody-associated vasculitis. Kidney involvement is one of the most common medical disorders, and the degree of renal damage is closely related to AAV's development and prognosis. We used the Robust Rank Aggregation technique in R to integrate GSE104948, GSE1081034, GSE1081092, GSE108112, and GSE1081132 profile datasets from the Gene Expression Omnibus database in this report, revealing a series of differentially expressed genes in kidney among AAV patients and living donors in this research. Both in glomerulus and tubulointerstitial, and intestinal, according to the results of gene ontology functional annotation in Then, immunity and metabolism were involved in the process of AAV both in glomerulus and tubulointerstitial. Both in glomerulus and tubulointerstitial compartments datasets, as well as S. aureus infections, according to a KEGG review.

Source link: https://europepmc.org/article/MED/35172670


Propylthiouracil-Induced Skin Vasculitis

The authors chronicle a case of a 61-year-old woman on PTU for one year who was referred to Internal Medicine for a three-month development of painless non-blanching purple patches, non-pruriginous, covering the chest and legs.

Source link: https://europepmc.org/article/MED/PMC9390865


Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS).

Methods We reviewed the medical records of 663 Asian patients with AAV, including 558 patients with recently diagnosed AAV and 105 with relapsed AAV. In 30 cases, including 20 with newly diagnosed AAV and ten with relapsed AAV, HP was found in the patients. In 50% of HP patients, Granulomatosis with polyangiitis was identified as a common cause. In patients with HP in total AAV and newly diagnosed AAV, a higher risk of GPA was much higher than in those without HP. In patients with HP and non-HP, serum proteinase 3 -ANCA positivity was significantly higher in patients with HP than in those without HP. Conclusion GPA was mainly observed in patients with HP. In addition, patients with HP in newly diagnosed AAV had significantly higher PR3-ANCA optimism than those without HP. The ear and eye regions can all be involved in HP formation.

Source link: https://europepmc.org/article/MED/35999568


Monocyte-derived macrophages aggravate pulmonary vasculitis via cGAS/STING/IFN-mediated nucleic acid sensing.

Patients suffering from anti-neutrophil cytoplasmic antibodies –associated vasculitis -associated vaping exhibited elevated cGAMP and elevated IFN-I signatures in this region. We created a mouse model for pulmonary AAV that mimics acute disease in patients in order to determine disease mechanisms and potential therapeutic targets. Residents' alveolar macrophages contributed to tissue homeostasis by clearing red blood cells and limiting infiltration of IFN-u03b2-producing macrophages, who were key disease generators by producing the majority IFN-u03b2-producing macrophages. Our research uncovers the importance of the STING/IFN-I axis in encouraging pulmonary AAV propagation and identifies cellular and molecular targets to reduce disease incidences.

Source link: https://europepmc.org/article/MED/35997679


Correct approach in urticarial vasculitis made early diagnosis of lupus nephritis possible: a case report.

Urticarial vasculitis is a clinicopathologic condition characterized by recurrent outbreaks of urticarial lesions that persist > 24 hours and reveal the histopathologic characteristics of leukocytoclastic vasculitis. Case report We present the case of a 61-year-old Caucasian woman with a skin eruption that resulted of erythematous plaques on the trunk and limbs that lasted more than 24 hours but were asymptomatic. Using Farr's analysis, there was also a positive antinuclear factor, with elevated U1 small nuclear ribonucleoprotein and high double-stranded DNA. With a full spectrum of immunoglobulin A, immunoglobulin G, and C1q, leading to lupus nephritis class III-A, the Kidney biopsy revealed focal crescent and focal mesangial type of glomerular injury with a fully developed positivity. Conclusion Clinicopathologic investigation of urticarial vasculitis with prompt testing for extracutaneous disease can lead to early diagnosis of significant organ involvement and therefore patient prognosis.

Source link: https://europepmc.org/article/MED/35989318


Arterial stiffness, endothelial dysfunction and impaired fibrinolysis are pathogenic mechanisms contributing to cardiovascular risk in ANCA-associated vasculitis.

Therefore, we compared arterial stiffness and endothelial function in patients with AAV in long-term disease remission and in matched healthy volunteers in a series of double-blind, randomized case-control forearm plethysmography, and crossover systemic interventional studies. The main outcome of the case-control study was the difference in endothelium-dependent vasodilation between health and AAV, and the difference in pulse wave velocity between placebo and selective endothelin-A receptor antagonism, which was the most important result for the crossover study, and the difference between treatment with placebo and selective endothelin-A receptor antagonism. Patients with AAV had significant arterial stiffness. Both selective endothelin-A/B receptor blockade and dual endothelin-A/B receptor blockade reduced PWV and increased tPA production in AAV in the crossover study relative to placebo. Patients with AAV in long-term remission have elevated cardiovascular risk, and endothelin-1 plays a role.

Source link: https://europepmc.org/article/MED/35998848


Vascular and Vasogenic Manifestations of Systemic ANCA-Associated Vasculitis with Renal Involvement in Non-Contrast Brain MRI in Patients with Acute Disease Onset.

The aim of the investigation was to investigate vascular and vasogenic brain changes in patients with acute onset of AAVR and the use of non-contrast magnetic resonance imaging techniques in this condition. Methods In the study, thirty-eight patients with acute onset of AAVR were included. In 94 percent of patients, SWAN was significantly more effective than the FLAIR method, according to the researchers. Conclusions Vascular and vasogenic changes in the CNS are common in patients with acute onset of systemic ANCA-associated vasculitis with renal involvement. The diagnosis of brain vasculitis is aided by non-contrast MRI.

Source link: https://europepmc.org/article/MED/36013102


Systemic Activation of Neutrophils by Immune Complexes Is Critical to IgA Vasculitis.

Polymorphonuclear neutrophil mediated damage is often traced to perivascular deposition of IgA1 immune complexes in IgA vasculitis. In patients and controls, IgA-IC levels and IgA-IC binding to PMNs were determined. During acute IgA-IC flares, we observed elevated serum IgA-ICs and increased IgA-IC binding to circulating PMNs. With the subsequent lowering of the threshold for NETosis, IgA-IC bound primed PMNs is shown by a substantial increase in NETs released from PMNs initiated in vitro and PMNs from IgAV patients with flares relative to surface IgA-negative PMNs after flares. In a murine model of IC vasculitis that significantly reduced vessel damage in vivo, inhibition of NET formation and knockout of myeloperoxidase decreased vessel damage in vivo. During active IgAV primes PMNs and vessel injury, increased adhesion of PMNs to the endothelium and increased NETosis, Binding of IgA-ICs.

Source link: https://europepmc.org/article/MED/35985788


Livedo Racemosa in Lymphocytic Vasculitis.

A 24-year-old female was born with a 10-year streak of mottled patches on the limbs. On direct immunofluorescence, Histology revealed a lymphocytic vasculitis with 1+ granular deposits of C3, IgM, and fibrin. This is connected to livedo racemosa, which is distinguished from livedo reticularis by the presence of larger broken rings that do not change with warming. Liveo racemosa is always pathological, unlike livedo reticularis, which may be physiological. A skin biopsy should include the center of the ring in order to determine the feeding artery, which is vitally.

Source link: https://europepmc.org/article/MED/35984296

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions