@vasculitides
@vasculitides.bsky.social
Living with EGPA/Churg-Strauss Syndrome • NIH-funded researcher • vasculitis patient advocate
www.vasculitides.com
www.vasculitides.com
Pinned
@vasculitides
@vasculitides.bsky.social
· Jan 21
Access our latest review on diagnosis, classification, and prognosis of ANCA-associated vasculitis in Nat Rev Rheum for free at rdcu.be/eL3wJ
Household-specific indoor PM(2.5) and PM(10-2.5) concentrations and Kawasaki disease onset by age six: the Japan Environment and Children's Study
Household-specific indoor PM2.5 and PM10-2.5 concentrations and Kawasaki disease onset by age six: the Japan Environment and Children's Study - PubMed
Higher household-specific indoor PM concentrations, particularly PM<sub>10-2.5</sub>, were associated with a lower risk of Kawasaki disease onset by 6 years of age. Further in-depth studies with larger sample sizes are needed.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:28 PM
Household-specific indoor PM(2.5) and PM(10-2.5) concentrations and Kawasaki disease onset by age six: the Japan Environment and Children's Study
Unraveling the Clinical Spectrum of DNASE1L3 Deficiency: Insights from Case Series and Systematic Literature Review
Unraveling the Clinical Spectrum of DNASE1L3 Deficiency: Insights from Case Series and Systematic Literature Review - PubMed
DNASE1L3 deficiency was associated with a broad clinical spectrum of immune-mediated disease rather than a single clinicopathological entity. The occurrence of identical pathogenic variants across distinct phenotypic and severity states argues against a simple genotype-phenotype model and suggests t …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:27 PM
Unraveling the Clinical Spectrum of DNASE1L3 Deficiency: Insights from Case Series and Systematic Literature Review
Benralizumab Effectiveness in EGPA
ClinicalTrials.gov
clinicaltrials.gov
September 12, 2026 at 11:25 PM
Benralizumab Effectiveness in EGPA
Oral Manifestations of Juvenile Behcet Syndrome: A Case Report and Mini-Review of the Literature
Oral Manifestations of Juvenile Behçet Syndrome: A Case Report and Mini-Review of the Literature - PubMed
Behçet's disease is a chronic, relapsing, multisystem inflammatory vasculitis characterized by a broad spectrum of mucocutaneous and systemic manifestations. Oral ulcerations are often the earliest and most frequent clinical feature, playing a central role in diagnosis, particularly in pediatric pat …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:25 PM
Oral Manifestations of Juvenile Behcet Syndrome: A Case Report and Mini-Review of the Literature
Predictive value of neutrophil-to-lymphocyte ratio and fibrinogen degradation products for renal involvement in pediatric immunoglobulin A vasculitis
Predictive value of neutrophil-to-lymphocyte ratio and fibrinogen degradation products for renal involvement in pediatric immunoglobulin A vasculitis - PubMed
Elevated NLR and FDP are independent risk factors for the development of IgAVN in patients with IgAV. The combination of NLR >5.23 and FDP >12.35 µg/mL demonstrates good clinical predictive value for IgAVN and warrants attention from clinicians.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:24 PM
Predictive value of neutrophil-to-lymphocyte ratio and fibrinogen degradation products for renal involvement in pediatric immunoglobulin A vasculitis
Metabolic reprogramming rewires aberrant immune responses to drive endothelial dysfunction in Kawasaki disease: a narrative review
Metabolic reprogramming rewires aberrant immune responses to drive endothelial dysfunction in Kawasaki disease: a narrative review - PubMed
This review highlights metabolic reprogramming as a key interface linking immune dysregulation, endothelial injury, and vascular complications in KD. Metabolic abnormalities may act not merely as consequences of inflammation but as active regulators of vascular dysfunction and disease progression. S …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:24 PM
Metabolic reprogramming rewires aberrant immune responses to drive endothelial dysfunction in Kawasaki disease: a narrative review
Integrated untargeted and targeted metabolomics combined with experimental validation reveal glutathione-related oxidative stress in pediatric IgA vasculitis nephritis
Integrated untargeted and targeted metabolomics combined with experimental validation reveal glutathione-related oxidative stress in pediatric IgA vasculitis nephritis - PubMed
Children with IgAVN exhibit a distinct metabolic profile characterized by glutathione depletion, activation of inflammation-related tryptophan metabolism, lipid/membrane remodeling, and disordered energy metabolism. Sphinganine, dehydroepiandrosterone, kynurenine, glutathione, nervonic acid, and cre …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:24 PM
Integrated untargeted and targeted metabolomics combined with experimental validation reveal glutathione-related oxidative stress in pediatric IgA vasculitis nephritis
Association of persistent oxidative stress markers with coronary artery abnormalities in Kawasaki disease patients on follow-up
Association of persistent oxidative stress markers with coronary artery abnormalities in Kawasaki disease patients on follow-up - PubMed
Children with KD exhibit persistent oxidative stress during follow-up, as evidenced by increased neutrophil ROS production, indicating ongoing cellular oxidative activity. Elevated ROS may serve as an accessible biomarker for CAAs and long-term cardiovascular risk in KD patients on follow-up.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:23 PM
Association of persistent oxidative stress markers with coronary artery abnormalities in Kawasaki disease patients on follow-up
Association of the Aggregate Index of Systemic Inflammation with coronary artery aneurysm in Kawasaki disease patients
Association of the Aggregate Index of Systemic Inflammation with coronary artery aneurysm in Kawasaki disease patients - PubMed
This study aimed to investigate the association between the Aggregate Index of Systemic Inflammation with coronary artery aneurysm in the acute phase Kawasaki disease. The Aggregate Index of Systemic Inflammation was closely associated with coronary artery aneurysm and served as a possible predictor …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:23 PM
Association of the Aggregate Index of Systemic Inflammation with coronary artery aneurysm in Kawasaki disease patients
Progress and Challenges in Kawasaki Disease: Increasingly International
Progress and Challenges in Kawasaki Disease: Increasingly International - PubMed
Progress and Challenges in Kawasaki Disease: Increasingly International
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:22 PM
Progress and Challenges in Kawasaki Disease: Increasingly International
Treatment and Outcomes of Kawasaki Disease Over 40 Years
Treatment and Outcomes of Kawasaki Disease Over 40 Years - PubMed
In this 40-year Italian cohort, coronary aneurysms were uncommon but exclusively determined long-term coronary sequelae. Younger age, delayed treatment or IVIG resistance, and pericardial effusion independently predicted aneurysm formation, highlighting major and potentially modifiable gaps in care …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:22 PM
Treatment and Outcomes of Kawasaki Disease Over 40 Years
Complement inhibition in pediatric ANCA-associated vasculitis
Complement inhibition in pediatric ANCA-associated vasculitis - PubMed
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in childhood is a rare but severe condition associated with significant morbidity and treatment-related toxicity. Current therapies mainly rely on high-dose glucocorticoids and conventional immunosuppressants, which may cause sub …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:21 PM
Complement inhibition in pediatric ANCA-associated vasculitis
Evaluation of Childhood Behcet's Disease: A Single-Center Experience
Evaluation of Childhood Behçet's Disease: A Single-Center Experience - PubMed
In childhood BD, mucocutaneous findings are the most common manifestations. The high frequency of family history and HLA-B51 positivity highlights genetic predisposition. Colchicine is effective as first-line therapy, with additional immunosuppressive agents used according to organ involvement.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:21 PM
Evaluation of Childhood Behcet's Disease: A Single-Center Experience
Comparison of Clinical and Laboratory Findings Between Skin-limited and Cutaneous-Articular Immunoglobulin A Vasculitis in Children
Comparison of Clinical and Laboratory Findings Between Skin-limited and Cutaneous-Articular Immunoglobulin A Vasculitis in Children - PubMed
Children with cutaneous-articular IgAV show a higher inflammatory profile than those with skin-limited disease. However, routine laboratory markers have limited utility for phenotypic discrimination. Despite higher ANA positivity in the cutaneous-articular phenotype, similar rates of additional rheu …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:21 PM
Comparison of Clinical and Laboratory Findings Between Skin-limited and Cutaneous-Articular Immunoglobulin A Vasculitis in Children
Association between choroidal vascularity index and disease activity in Behcet uveitis using BOS24 and FFA scores
Association between choroidal vascularity index and disease activity in Behçet uveitis using BOS24 and FFA scores - PubMed
CVI was lower in Behçet uveitis despite comparable choroidal thickness, indicating alterations in choroidal luminal-stromal composition that were not reflected by thickness measurements alone. Its relationship with BOS24-defined clinical activity was more evident than that with FFA-defined activity. …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:20 PM
Association between choroidal vascularity index and disease activity in Behcet uveitis using BOS24 and FFA scores
Baseline comorbidity assessment may authorize early steroid-sparing therapy in older patients with polymyalgia rheumatica
Baseline comorbidity assessment may authorize early steroid-sparing therapy in older patients with polymyalgia rheumatica - PubMed
Glucocorticoids remain the cornerstone of polymyalgia rheumatica (PMR) management, but prolonged use carries significant toxicity risks in older adults. Despite high multi-morbidity prevalence, systematic screening using validated geriatric scales is rarely performed. We evaluated whether baseline s …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:20 PM
Baseline comorbidity assessment may authorize early steroid-sparing therapy in older patients with polymyalgia rheumatica
Multi-database pharmacovigilance identifies disproportionate reporting of hepatobiliary events with avacopan: an integrative study with network pharmacology and interpretable machine learning
Multi-database pharmacovigilance identifies disproportionate reporting of hepatobiliary events with avacopan: an integrative study with network pharmacology and interpretable machine learning - PubMed
Avacopan is an oral C5a receptor antagonist approved for severe active granulomatosis with polyangiitis and microscopic polyangiitis. However, its real-world safety profile, particularly hepatobiliary safety signals, remains incompletely characterized. We analyzed avacopan-related adverse event repo …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:19 PM
Multi-database pharmacovigilance identifies disproportionate reporting of hepatobiliary events with avacopan: an integrative study with network pharmacology and interpretable machine learning
Takayasu Arteritis in a G6PD Deficient Female Patient With Recurrent Axillary Artery Stenosis: A Case Report
Takayasu Arteritis in a G6PD Deficient Female Patient With Recurrent Axillary Artery Stenosis: A Case Report - PubMed
Takayasu arteritis should be suspected in young females with limb claudication, absent pulses, recurrent arterial stenosis, and elevated inflammatory markers. Early diagnosis is crucial to prevent com...
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:19 PM
Takayasu Arteritis in a G6PD Deficient Female Patient With Recurrent Axillary Artery Stenosis: A Case Report
Untargeted metabolomics reveals differential metabolic pathways and biomarkers in the acute phase of Kawasaki disease
Untargeted metabolomics reveals differential metabolic pathways and biomarkers in the acute phase of Kawasaki disease - PubMed
Untargeted metabolomics enables identification of metabolically perturbed pathways during the acute phase of KD. L-tyrosine, L-tryptophan, glutamine, histidine, histamine, and taurocholic acid may serve as candidate biomarkers for acute phase of KD.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:18 PM
Untargeted metabolomics reveals differential metabolic pathways and biomarkers in the acute phase of Kawasaki disease
Cytokine-augmented risk stratification for six-month incident nephritis in children with IgA vasculitis
Cytokine-augmented risk stratification for six-month incident nephritis in children with IgA vasculitis - PubMed
In this single-center derivation cohort, CAS improved internally evaluated discrimination, Brier score, and risk separation beyond clinical variables and RIIS. The model remains investigational pending external validation, assay standardization, and implementation testing.
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:18 PM
Cytokine-augmented risk stratification for six-month incident nephritis in children with IgA vasculitis
Heart failure and predictors of outcome in eosinophilic myocarditis: a real-world cohort study
Heart failure and predictors of outcome in eosinophilic myocarditis: a real-world cohort study - PubMed
In this real-world cohort, EM severely impacted multiple domains of cardiac function. Treatment patterns were heterogeneous. Baseline NT-proBNP, LVEF, and the Sartorelli score were associated with long-term LVEF and LV remodeling. Baseline evidence of active myocardial inflammation on CMR and/or EMB …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:17 PM
Heart failure and predictors of outcome in eosinophilic myocarditis: a real-world cohort study
Subglottic stenosis in granulomatosis with polyangiitis: clinical, histopathological and therapeutic considerations with presentation of two cases
Subglottic stenosis in granulomatosis with polyangiitis: clinical, histopathological and therapeutic considerations with presentation of two cases - PubMed
Granulomatosis with polyangiitis (GPA), once known as Wegener's granulomatosis (WG), is an uncommon systemic vasculitic disorder characterized by inflammation of the vascular walls, particularly affecting the respiratory tract. One serious complication is subglottic stenosis (SGS), a narrowing of th …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 11:17 PM
Subglottic stenosis in granulomatosis with polyangiitis: clinical, histopathological and therapeutic considerations with presentation of two cases
Vasculitis Syndromes with Renal Involvement: A Review from a Case Series
Vasculitis Syndromes with Renal Involvement: A Review from a Case Series - PubMed
This review describes vasculitis syndromes that can be diagnosed by kidney biopsy. Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) typically follow a rapidly progressive course, often leading to end-stage renal failure within a few months, with crescentic glomerulonephritis …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 12:37 AM
Vasculitis Syndromes with Renal Involvement: A Review from a Case Series
A Diagnostic Approach to Giant Cell Arteritis Initially Presenting with Infarction without Headache Using MR Vessel Wall Imaging and a Temporal Artery Biopsy: A Case Report
A Diagnostic Approach to Giant Cell Arteritis Initially Presenting with Infarction without Headache Using MR Vessel Wall Imaging and a Temporal Artery Biopsy: A Case Report - PubMed
A 75-year-old woman presented with a sudden onset of left homonymous hemianopia and dizziness without headache. Magnetic resonance imaging revealed multiple posterior circulation infarctions, and magnetic resonance angiography revealed right vertebral artery occlusion and left vertebral artery steno …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 12:36 AM
A Diagnostic Approach to Giant Cell Arteritis Initially Presenting with Infarction without Headache Using MR Vessel Wall Imaging and a Temporal Artery Biopsy: A Case Report
Discordance between disease activity and long-term outcomes in DNASE1L3 deficiency: a multicentre longitudinal cohort study
Discordance between disease activity and long-term outcomes in DNASE1L3 deficiency: a multicentre longitudinal cohort study - PubMed
In the largest DNASE1L3 deficiency cohort, reduction in clinical disease activity does not prevent progressive organ damage. These findings suggest that apparent disease quiescence may mask ongoing subclinical injury, underscoring the need for sustained long-term surveillance and optimised transitio …
pubmed.ncbi.nlm.nih.gov
September 12, 2026 at 12:34 AM
Discordance between disease activity and long-term outcomes in DNASE1L3 deficiency: a multicentre longitudinal cohort study