Higher household-specific indoor PM concentrations, particularly PM_10-2.5, 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.

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 …

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 …

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.

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 …

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 …

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.

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 …

Progress and Challenges in Kawasaki Disease: Increasingly International

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 …

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 …

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.

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 …

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. …

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 …

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 …

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...

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.

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.

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 …

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 …

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 …

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 …

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 …

Response to 'Correspondence on "Results of a 1-year randomized double-blind placebo-controlled trial with methotrexate 25 mg/week in recently diagnosed polymyalgia rheumatica" by Bolhuis TE et al'

Correspondence on 'Results of a 1-year randomised double-blind placebo-controlled trial with methotrexate 25 mg/week in recently diagnosed polymyalgia rheumatica' by Bolhuis et al

Th17/Treg imbalance is an immunopathological feature of HSP. hUC-MSCs restore immune homeostasis by inhibiting STAT3 signaling, highlighting STAT3 as a potential therapeutic target and supporting further investigation of stem cell-based therapy.

AAV hospital burden varied substantially across jurisdictions in rates, subtype distribution, and outcomes, warranting cautious interpretation in light of differences in population structure and health system capture.

This first national cohort from North Macedonia confirms that AAV with renal involvement presents with advanced kidney dysfunction, a compressed time to RRT, and substantial early mortality. Baseline renal function remains the dominant determinant of renal outcome, underscoring the need for earlier …

The majority of ANCA-positive patients did not have AAV. In our secondary care setting, ELISA performs best as first test to rule out and to rule in AAV. A second test should be considered with high clinical suspicion and a negative first test, or with low antibody concentrations, following the cons …

TPE is selectively used as an adjunctive therapy in children with severe or refractory AAV, particularly in those with advanced kidney involvement. Although disease activity and acute organ-threatening manifestations improved following TPE, persistent renal abnormalities remained frequent, reflectin …

Upadacitinib represents a potential therapeutic option for patients with anti-TNF-refractory intestinal BD.

Combined therapy with eculizumab showed improved early kidney survival for anti-GBM nephritis at 12 weeks, and did not increase the risk of adverse events. Prospective, large-sample studies with long-term follow-up are needed to validate its efficacy and safety.

Among large Japanese and US cohorts, coronary artery dilation at diagnosis and age younger than 6 months were consistent predictors of CAA. These associations were also observed for medium to giant CAA, underscoring their relevance for clinically significant coronary outcomes. Early coronary artery …

Rapid Steroid-free Clearance of Refractory Leukocytoclastic Vasculitis With Abrocitinib

Transition of care for adolescents and young adults with Kawasaki disease: An international survey

Relapsing polychondritis is a rare chronic autoimmune inflammation of the cartilage associated with life-threatening respiratory complications. Currently, no clear role of imaging modalities such as 18F-fluorodeoxyglucose (FDG) PET/computed tomography (CT) is defined in the literature. This systemat …

Recurrent Kawasaki Disease with Delayed-Onset Coronary Artery Dilatation and Late Normalization

• Baseline NT-proBNP was independently associated with KDSS, and age-adjusted NT-proBNP z-scores showed comparable predictive performance, supporting NT-proBNP as an adjunctive marker for early KDSS risk stratification after consideration of age dependency.

One in seven people with ANCA-associated vasculitis has kidney artery inflammation, which can boost the risk of kidney failure, per a study.

Kidney Disease: Improving Global Outcomes (KDIGO) responds to the retraction of the ADVOCATE trial of avacopan for the treatment of ANCA-associated vasculitis

ANCA fluorescence patterns provide complementary diagnostic information, whereas formalin-fixed substrate testing adds limited value when reliable MPO-ANCA assays are available. An LR-based framework integrating antibody levels and fluorescence patterns may improve the interpretation of ANCA results …

Anterior ischemic optic neuropathy (AION) is one of the leading causes of sudden, painless, monocular vision loss in individuals over 50 years of age. Two forms can be distinguished, with clearly different clinical, diagnostic, and therapeutic implications: the arteritic form, most commonly associat …

Collateral vessels supplying blood to the brain and the entire upper body in a patient with Takayasu arteritis

Giant cell arteritis developed under secukinumab treatment

The combined assessment of tympanic membrane findings using the SCOT score and the bone conduction threshold at 500 Hz may provide useful otologic clues that prompt further evaluation for OMAAV, including ANCA testing, in elderly patients with refractory OME.

Rh-irAEs in a rheumatologists-referred population were more severe than previously described, with frequent ICI discontinuation and escalation beyond GCs. Male sex was associated with treatment escalation. An underrecognized axSpA-like phenotype occurred in younger patients, warranting further study …

Glucocorticoids remain among the most widely prescribed immunosuppressive and anti-inflammatory agents worldwide, yet their clinical use is complicated by substantial inter-individual variability in both therapeutic response and adverse effects. Despite decades of clinical experience, glucocorticoid …

Immune-mediated nephropathies arise from the interaction between systemic immune dysregulation and a nephron-specific immune microenvironment. Distinguishing the shared immune programs from disease-defining cascades provides a rational basis for stratification and precision medicine. Integrated sero …