4,584 results match your criteria vasculitis healthy


Hygiene Hypothesis as the Etiology of Kawasaki Disease: Dysregulation of Early B Cell Development.

Authors:
Jong-Keuk Lee

Int J Mol Sci 2021 Nov 15;22(22). Epub 2021 Nov 15.

Asan Medical Center, Asan Institute for Life Sciences, University of Ulsan College of Medicine, Seoul 05505, Korea.

Kawasaki disease (KD) is an acute systemic vasculitis that occurs predominantly in children under 5 years of age. Despite much study, the etiology of KD remains unknown. However, epidemiological and immunological data support the hygiene hypothesis as a possible etiology. Read More

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November 2021

The Network of Pro-Inflammatory Factors CD147, DcR3, and IL33 in the Development of Kawasaki Disease.

J Inflamm Res 2021 19;14:6043-6053. Epub 2021 Nov 19.

Department of Cardiology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, 310052, People's Republic of China.

Introduction: Kawasaki disease (KD) is an acute febrile systemic vasculitis, but the etiology remains unknown. We studied serum levels of CD147, DcR3, and IL33 in different stages of KD to explore the value of CD147, DcR3, and IL33 in the pathophysiology of KD.

Methods: We measured serum levels of CD147, DcR3, and IL33 by enzyme-linked immunosorbent assay (ELISA) at different stages with 71 KD patients and 66 healthy control children. Read More

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November 2021

Elevated Myeloperoxidase-DNA Complex Levels in Sera of Patients with IgA Vasculitis.

Pathobiology 2021 Nov 23:1-6. Epub 2021 Nov 23.

Department of Dermatology, St. Marianna University School of Medicine, Kawasaki, Japan.

Introduction: IgA vasculitis is a systemic disease that results from the entrapment of circulating IgA-containing immune complexes in small-vessel walls in the skin, kidneys, and gastrointestinal tract. An excessive formation of neutrophil extracellular traps (NETs) is involved in the pathogenesis of vasculitis, especially in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This study aimed to clarify whether NETs are implicated in IgA vasculitis. Read More

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November 2021

Epidemiology and Clinical Characteristics of Henoch-Schönlein Purpura Associated with Epstein-Barr Virus Infection.

Mediterr J Hematol Infect Dis 2021 1;13(1):e2021064. Epub 2021 Nov 1.

Medical department, The first people's Hospital of Guangshui, China.

Background: Henoch-Schönlein purpura (HSP) is an immune-mediated vasculitis, and the formation of immune complexes may be triggered by exposure to Epstein-Barr virus (EBV) infection.

Methods: We performed a five-year case-control study to evaluate the epidemiology and clinical characteristics of HSP associated with EBV infection.

Results: The incidence of EBV-triggered HSP was 4. Read More

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November 2021

ANCA vasculitis: A manifestation of Post-Covid-19 Syndrome.

Respir Med Case Rep 2021 11;34:101549. Epub 2021 Nov 11.

Department of Internal Medicine, Kirk Kerkorian School of Medicine at UNLV, USA.

The SARS-CoV-2 infection has been found to present with different degrees of response and variable levels of inflammation. Patients who have recovered from the initial infection can develop long-term symptomatology. We present a unique case of a middle aged-healthy man who developed complications of ANCA-associated vasculitis after recovering from a mild COVID-19 infection. Read More

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November 2021

Relationship between Gd-IgA1 and TNFR1 in IgA nephropathy and IgA vasculitis nephritis in children - multicenter study.

Cent Eur J Immunol 2021 7;46(2):199-209. Epub 2021 Aug 7.

Department of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, Poland.

Aim Of The Study: To evaluate the relationship between serum Gd-IgA1 (sGd-IgA1) and serum and urine TNFR1 (sTNFR1, uTNFR1) levels as possible prognostic factors in IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN).

Material And Methods: From 299 patients from the Polish Registry of Pediatric IgAN and IgAVN, 60 children (24 IgAN and 36 IgAVN) were included in the study. The control group consisted of 20 healthy children. Read More

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Imbalanced Th17/Treg in peripheral blood of adult patients with immunoglobulin A vasculitis nephritis.

Cent Eur J Immunol 2021 7;46(2):191-198. Epub 2021 Aug 7.

Hubei Provincial Hospital of Traditional Chinese Medicine, Wuhan, Hubei, China.

Introduction: Adult immunoglobulin A vasculitis nephritis (IgAVN) was observed to be more severe than the disease in children because it tended to result in a poor prognosis. The present study analyzed the Th17/Treg cell axis in peripheral blood of adult IgAVN patients, aiming to provide new immunological viewpoints for the pathogenesis of adult IgAVN.

Material And Methods: Th17 cell and Treg cell frequencies in peripheral blood of healthy subjects (n = 13) and adult IgAVN patients (n = 12) were analyzed by flow cytometry. Read More

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Histopathology of new-onset refractory status epilepticus (NORSE) in adults.

Seizure 2021 Oct 5;93:95-101. Epub 2021 Oct 5.

Virginia Tech Carilion School of Medicine, Roanoke, VA, United States; Carilion Clinic Neurology, Roanoke, VA, United States.

Objective: new-onset refractory status epilepticus (NORSE) is defined as de novo refractory seizures occurring in previously healthy adults, without a clear underlying etiology. Due to refractory seizures and insufficient understanding of pathophysiology, management of these patients remains challenging and often leads to poor clinical outcomes. Various infectious and autoimmune mechanisms have been proposed but have not been validated and a large number of patients are thus labeled 'cryptogenic'. Read More

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October 2021

Polymorphisms Confer Risk of Anti-CCP-Positive Rheumatoid Arthritis in Synergy With and Smoking.

Front Immunol 2021 18;12:707690. Epub 2021 Oct 18.

Institute for Inflammation Research, Center for Rheumatology and Spine Diseases, Section 7521, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.

Peptidylarginine deiminases (PADs) catalyze citrullination, a post-translational modification playing a pathogenic role in anti-citrullinated protein antibody (ACPA)-positive rheumatoid arthritis (RA). The interplay between single nucleotide polymorphisms (SNPs) in the genes and known risk factors for ACPA-positive RA, including smoking, HLA-DR4 and -1, and the PTPN22 R620W polymorphism, was investigated. We typed four SNPs, four SNPs, and the PTPN22 R620W SNP in 445 Danish RA patients and 533 age-matched healthy controls, as well as in 200 North American RA patients and 100 age- and sex-matched controls. Read More

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October 2021

Plasma Pyruvate Kinase M2 as a marker of vascular inflammation in Giant Cell Arteritis.

Rheumatology (Oxford) 2021 Nov 3. Epub 2021 Nov 3.

Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Objectives: Giant Cell Arteritis (GCA) is a large vessel vasculitis in which metabolically active immune cells play an important role. GCA diagnosis is based on CRP/ESR and temporal artery biopsies (TABs), in combination with [18F]FDG-PET/CT relying on enhanced glucose uptake by glycolytic macrophages. Here, we studied circulating Pyruvate Kinase M2 (PKM2), a glycolytic enzyme, as a possible systemic marker of vessel wall inflammation in GCA. Read More

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November 2021

Single-cell profiling of T lymphocytes in deficiency of adenosine deaminase 2.

J Leukoc Biol 2021 Nov 3. Epub 2021 Nov 3.

Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA.

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic vasculitis syndrome caused by autosomal-recessive loss-of-function mutations in the ADA2 gene (previously known as CECR1). Vasculitis, vasculopathy, and inflammation are dominant clinical features of this disease; the spectrum of manifestations includes immunodeficiency and lymphoproliferation as well as hematologic manifestations. ADA2 is primarily secreted by stimulated monocytes and macrophages. Read More

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November 2021

Kawasaki disease and increased cardiovascular risk: Is there a link to circulating glycocalyx biomarkers?

Microvasc Res 2021 Oct 23;140:104269. Epub 2021 Oct 23.

Ludwig-Maximilians-University of Munich, Department of Pediatric Cardiology, Marchioninistr. 15, 81377 Munich, Germany.

Aims: Kawasaki disease (KD) is an acute systemic vasculitis with possible long-term impact of general cardio-vascular health. An endothelial glycocalyx disorder during the disease's acute phase might predispose to long-term vascular anomalies leading to endothelial dysfunction and atherosclerosis. To investigate any association between increased cardiovascular risk and endothelial glycocalyx, we assessed circulating glycocalyx components in patients with a KD history, and analysed their association with acute-phase clinical features and more importantly, with patients' current cardiovascular risk factors. Read More

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October 2021

Henoch-Schönlein Purpura Following the First Dose of COVID-19 Viral Vector Vaccine: A Case Report.

Vaccines (Basel) 2021 Sep 25;9(10). Epub 2021 Sep 25.

Department of Life, Health and Environmental Sciences, University of L'Aquila, 67100 L'Aquila, Italy.

A 76 year-old female came to our observation one week after the vaccination with ChAdOx1 nCoV-19 AZD1222 for the onset of purpuric rash on her gluteal and legs regions associated with coxalgia and episodes of macrohaematuria. Henoch-Schönlein purpura (HSP) was diagnosed on the basis of the revised criteria developed by the European League Against Rheumatism, the Paediatric Rheumatology International Trials Organization, and the Paediatric Rheumatology European Society (EULAR/PRINTO/PRES). HSP is a common IgA-mediated small vessel vasculitis, typical of childhood, that affects several systems and is characterized by a tetrad of dermatological, abdominal, joint, and renal manifestations. Read More

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September 2021

Serum Clusterin Level Could Reflect the Current Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Yonsei Med J 2021 Nov;62(11):1016-1022

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

Purpose: We investigated whether serum clusterin levels could reflect the current antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-specific indices.

Materials And Methods: Fifty-seven patients with AAV and 40 healthy controls were included in this study. AAV-specific indices included the Short-Form 36-Item Health Survey Physical and Mental Component Summaries (SF-36 PCS and MCS) scores, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), and vasculitis damage index. Read More

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November 2021

When obstetrics-gynecology specialists need to call an ophthalmologist urgently: a case report.

J Med Case Rep 2021 Oct 21;15(1):517. Epub 2021 Oct 21.

Service de Gynécologie et Obstétrique, Département Femme-Mère-Enfant, Centre Hospitalier Universitaire Vaudois, CHUV, 1011, Lausanne, Switzerland.

Background: We report here a case of a healthy 23-year-old female patient who was assessed at the gynecology emergency department for genital ulcers, fever, and blurred vision. After suspicion of herpes simplex virus-2 lesions, the diagnosis of Behçet's disease was made. We report this case with the aim of including Behçet's disease in the differential diagnosis of genital ulcers, and emphasize the emergency of the vision loss that can be irreversible. Read More

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October 2021

Autoantibodies against the envelope proteins of endogenous retroviruses K102 and K108 in patients with systemic lupus erythematosus correlate with active disease.

Clin Exp Rheumatol 2021 Oct 13. Epub 2021 Oct 13.

Division of Rheumatology, Department of Medicine, University of Washington, Seattle, WA, USA.

Objectives: To determine if patients with systemic lupus erythematosus (SLE), a disease characterised by elevated type I interferons reminiscent of anti-viral immunity, have expression of human endogenous retrovirus K (HERV-K) proviruses capable of producing envelope (Env) protein, as well as associated autoantibodies against the Env protein.

Methods: ELISAs were conducted with recombinant Env protein and sera from SLE patients with active (n=60) or inactive (n=49) disease, healthy controls (n=47), other rheumatic disorders (n=59), as well as plasma from paediatric lupus patients with active (n=30) or inactive (n=30) disease, and 17 healthy children. Antibody reactivity was evaluated for correlations with clinical and laboratory parameters of the patients. Read More

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October 2021

Metagenomic profiling reveals dominance of gram-positive bacteria in the gut microbiome shifts associated with immunoglobulin A vasculitis (Henoch-Schönlein Purpura).

Clin Transl Immunology 2021 8;10(10):e1342. Epub 2021 Oct 8.

Department of Pediatrics The First Affiliated Hospital of Kunming Medical University Kunming China.

Objectives: Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common vasculitis that has a classical skin manifestation of palpable purpuric rash. Factors pertinent to IgAV remain inadequately understood. Here, we aimed to examine the gut microbiome shifts associated with IgAV and its recovery. Read More

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October 2021

Potential Utility of Oral Mucosal Capillaroscopy as an Indicator of Microvascular Damage in Behçet Disease: A Preliminary Study.

Dermatol Pract Concept 2021 Sep 1;11(4):e2021116. Epub 2021 Sep 1.

Department of Dermatology, Guglielmo Marconi University, Rome, Italy.

Introduction: Behçet disease (BD) is an auto-inflammatory condition characterized by multisystemic vasculitis. Oral mucosal capillaroscopy is an easy-to-use, repeatable, non-invasive method for evaluating mucosal microvasculature, contributing to the differential diagnosis and prognosis of various acute and chronic inflammatory diseases.

Objectives: This study aims to characterize and describe the oral labial mucosal capillary findings in patients with BD using handheld dermatoscopy and to investigate the relationship between the capillary findings and the severity of the disease. Read More

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September 2021

Immune checkpoint molecule expression is altered in the skin and peripheral blood in vasculitis.

Sci Rep 2021 Oct 8;11(1):20019. Epub 2021 Oct 8.

Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

Dysfunction of immunoinhibitory signals and persistent T cell activation reportedly play important roles in the development of vasculitis. The skin is one of the most accessible organs, and it is suitable for the characterization of immune cell signatures. However, the inhibitory checkpoint molecules in the skin and their relevance to vasculitis have not been studied. Read More

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October 2021

Circulating autoreactive proteinase 3+ B cells and tolerance checkpoints in ANCA-associated vasculitis.

JCI Insight 2021 Nov 22;6(22). Epub 2021 Nov 22.

Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA.

BACKGROUNDLittle is known about the autoreactive B cells in antineutrophil cytoplasmic antibody-associated (ANCA-associated) vasculitis (AAV). We aimed to investigate tolerance checkpoints of circulating antigen-specific proteinase 3-reactive (PR3+) B cells.METHODSMulticolor flow cytometry in combination with bioinformatics and functional in vitro studies were performed on baseline samples of PBMCs from 154 well-characterized participants of the RAVE trial (NCT00104299) with severely active PR3-AAV and myeloperoxidase-AAV (MPO-AAV) and 27 healthy controls (HCs). Read More

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November 2021

Avascular necrosis of the first metatarsal head in a young female adult: A case report and review of literature.

World J Clin Cases 2021 Sep;9(25):7445-7452

Department of Orthopaedics and Traumatology, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong 999077, China.

Background: This case study describes an atypical presentation of avascular necrosis (AVN) of the first metatarsal head, which is largely unfounded in the literature.

Case Summary: A healthy 24-year-old female initially presented with pain at the first metatarsophalangeal joint (MTPJ) and was diagnosed with AVN by physical examination and magnetic resonance imaging. The patient demonstrated atypically poor progress in recovery, despite being in otherwise good health and being of young age, with no history of corticosteroid or alcohol use. Read More

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September 2021

Vascular Abnormalities in Peripapillary and Macular Regions of Behcet's Uveitis Patients Evaluated by Optical Coherence Tomography Angiography.

Front Med (Lausanne) 2021 16;8:727151. Epub 2021 Sep 16.

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China.

To investigate the involvement of peripapillary zone vascular abnormalities in Behcet's uveitis (BU) and associated visual dysfunction. We evaluated the retinal and choroidal microvascular features in both macular and peripapillary areas of BU patients to identify vascular abnormalities contributing to reduced best-corrected visual acuity (BCVA) using optical coherence tomography angiography (OCTA). A prospective, observational study was conducted in 24 eyes of 13 patients with BU and 24 eyes of 15 healthy participants as controls. Read More

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September 2021

Initial Presentation of Granulomatosis with Polyangiitis as Progressive Skull Base Osteomyelitis.

ORL J Otorhinolaryngol Relat Spec 2021 Sep 30:1-5. Epub 2021 Sep 30.

Department of Otolaryngology-Head and Neck Surgery, University of Washington, Seattle, Washington, USA.

A healthy man in his 30s presented with a 2-week history of severe bitemporal pain and pressure. He was initially treated for presumed acute rhinosinusitis, but his symptoms continued to worsen and underwent endoscopic sinus surgery at an outside community facility. He developed left abducens nerve palsy postoperatively, and magnetic resonance imaging (MRI) demonstrated evidence of extensive skull base osteomyelitis. Read More

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September 2021

Novel aspects of regulatory T cell dysfunction as a therapeutic target in giant cell arteritis.

Ann Rheum Dis 2021 Sep 28. Epub 2021 Sep 28.

Rheumatology and Immunology, Hannover Medical School, Hannover, Germany

Objectives: Giant cell arteritis (GCA) is the most common primary vasculitis, preferentially affecting the aorta and its large-calibre branches. An imbalance between proinflammatory CD4 T helper cell subsets and regulatory T cells (Tregs) is thought to be involved in the pathogenesis of GCA and Treg dysfunction has been associated with active disease. Our work aims to explore the aetiology of Treg dysfunction and the way it is affected by remission-inducing immunomodulatory regimens. Read More

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September 2021

Serum ANCA and Overall Mortality: A 10-Year Retrospective Cohort Study on 1,024 Italian Subjects.

Front Immunol 2021 10;12:714174. Epub 2021 Sep 10.

Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy.

Background: Antineutrophil cytoplasmic antibodies (ANCA) are primarily involved in the pathogenesis of ANCA-associated vasculitides (AAV). However, ANCA may also be present in healthy subjects and in patients with autoimmune disorders different from AAV. We hypothesized that serum ANCA are associated with a worse prognosis in disorders other than AAV. Read More

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September 2021

Neutrophil-mediated mechanisms of damage and in-vitro protective effect of colchicine in non-vascular Behçet's syndrome.

Clin Exp Immunol 2021 Dec 8;206(3):410-421. Epub 2021 Oct 8.

Department of Experimental and Clinical Biomedical Sciences 'Mario Serio', University of Firenze, Firenze, Italy.

Behçet's syndrome (BS) is a systemic vasculitis with several clinical manifestations. Neutrophil hyperactivation mediates vascular BS pathogenesis, via both a massive reactive oxygen species (ROS) production and neutrophil extracellular traps (NETs) release. Here, we investigated neutrophil-mediated mechanisms of damage in non-vascular BS manifestations and explored the in-vitro effects of colchicine in counteracting these mechanisms. Read More

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December 2021

The Involvement of Neutrophil Extracellular Traps in Disease Activity Associated With IgA Vasculitis.

Front Immunol 2021 3;12:668974. Epub 2021 Sep 3.

Department of Pediatrics, The First Affiliated Hospital, Guangxi Medical University, Nanning, China.

Objectives: This aim of this study was to determine whether neutrophil extracellular traps (NETs) are involved in the pathogenesis of IgA vasculitis (IgAV) and investigate whether the circulating NETs levels are associated with disease activity in children.

Methods: We performed a case-control study and collected blood samples from 193 children with different stages of IgAV (61 were at the onset stage, 64 at the remission stage, 43 at the active stage, and 25 were undergoing drug withdrawal). A total of 192 healthy children were recruited as controls. Read More

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October 2021

IgG anti-Pentraxin 3 antibodies are a novel biomarker of ANCA-associated vasculitis and better identify patients with eosinophilic granulomatosis with polyangiitis.

J Autoimmun 2021 Nov 14;124:102725. Epub 2021 Sep 14.

Division of Rheumatology, Department of Medicine DIMED, University of Padova, Italy. Electronic address:

Objective: To investigate prevalence of anti-Pentraxin 3 (PTX3) antibodies in sera of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) patients.

Methods: Anti-PTX3 and PTX3 levels were analysed by enzyme-linked immunosorbent assays in sera from unselected patients with AAV and compared with patients with systemic lupus erythematosus (SLE, n = 130), other connective tissue diseases (CTDs, n = 97) and matched healthy controls (n = 97). Optical density (OD) cut-off for positive anti-PTX3 antibodies was determined by ROC curve analysis and set as 0. Read More

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November 2021

Variations of tongue coating microbiota in children with Henoch-Schönlein purpura nephritis.

Microb Pathog 2021 Nov 14;160:105192. Epub 2021 Sep 14.

Department of Nephrology, Zhongshan Hospital, Fudan University; Shanghai Institute of Kidney and Dialysis; Shanghai Key Laboratory of Kidney and Blood Purification; Shanghai Medical Center of Kidney Disease, Shanghai, 200433, China. Electronic address:

Background: Variations in the oral microbiota have been significantly correlated with the progress of autoimmune diseases, such as immunoglobulin A nephropathy and Henoch-Schönlein purpura (HSP). However, there is no report outlining the character of tongue coating microbiota variations in children with Henoch-Schönlein purpura nephritis (HSPN).

Method: A total of 20 children with HSPN and 14 healthy controls were recruited for this research. Read More

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November 2021

Distribution of monocytes subpopulations in the peripheral blood from patients with Behçet's disease - Impact of disease status and colchicine use.

Clin Immunol 2021 10 13;231:108854. Epub 2021 Sep 13.

Rheumatology Division, Department of Medicine, Universidade Federal de São Paulo - Escola Paulista de Medicina, Rua Botucatu, 740, 04023-900 São Paulo, SP, Brazil. Electronic address:

The innate immune response has a predominant role in Behçet's disease (BD) pathogenesis, but few studies have assessed monocytes in BD. This study aims to evaluate the profile of monocytes subsets in the peripheral blood of BD patients and healthy controls (HC). Monocytes subsets were identified as classical (CD14CD16), intermediate (CD14CD16), and non-classical (CD14CD16) subsets. Read More

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October 2021