[This corrects the article DOI: 10.3389/fimmu.2025.1742328.].

Cryoglobulinemia (CG) is a rare disease characterized by the presence of circulating immunoglobulins that precipitate at low temperatures and may lead to systemic manifestations. We report the case of a 73-year-old woman who met the diagnostic criteria for type I cryoglobulinemic vasculitis, presenting with ulceronecrotic, partly purpuric lesions. Skin biopsy revealed vasculitis, and laboratory evaluation revealed an IgG kappa monoclonal gammopathy, leading to multiple myeloma (MM) as the underlying etiology. Clone-directed therapy resulted in marked clinical improvement, with significant regression of cutaneous lesions within two months. Given its rarity and potential for severe complications, this case highlights the importance of systematically investigating an underlying monoclonal gammopathy in patients presenting with type I cryoglobulinemic vasculitis.

Behçet's disease is a complex, multisystem inflammatory vasculitis of unknown etiology, characterized by a highly heterogeneous clinical presentation that may delay recognition, particularly in the emergency setting. We report a case of a 15-year-old girl presenting to the emergency department with a first episode of painful genital ulceration associated with erythema nodosum and persistent fever. Laboratory investigations revealed a marked inflammatory syndrome without an identifiable infectious cause. Based on clinical findings and according to the International Criteria for Behçet's Disease (ICBD), a score of 5 was obtained, supporting the diagnosis. This case underscores the diagnostic challenges associated with early-stage Behçet's disease, especially in young patients presenting with incomplete or atypical clinical features. It highlights the importance of maintaining a high index of suspicion in the emergency setting to facilitate timely diagnosis and appropriate management.

Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) are pediatric inflammatory conditions with significant cardiovascular involvement, particularly affecting the coronary arteries. KD remains the leading cause of acquired heart disease in children worldwide, while MIS-C has emerged as a postinfectious complication of severe acute respiratory syndrome coronavirus 2 with overlapping but distinct cardiovascular manifestations. Coronary artery abnormalities, including dilation and aneurysm formation, represent the most important cardiovascular sequelae in KD, whereas coronary involvement in MIS-C appears less frequent and often transient, although its long-term implications remain incompletely understood. Early detection and longitudinal assessment of coronary involvement are essential for optimizing outcomes. Echocardiography is the first-line imaging modality for both KD and MIS-C due to its accessibility and high sensitivity in detecting proximal coronary abnormalities. However, advanced imaging techniques such as coronary computed tomography angiography and cardiac magnetic resonance imaging provide superior visualization of distal coronary segments, vessel wall characteristics, myocardial perfusion, and fibrosis. These modalities are increasingly integrated into follow-up strategies, especially in patients with complex or persistent lesions. Risk stratification is guided by coronary artery Z scores, inflammatory biomarkers, and clinical severity, enabling tailored therapeutic approaches, including antiplatelet and anticoagulation therapy. Despite advances in treatment, a subset of patients with KD develops persistent coronary artery abnormalities with potential progression to ischemic heart disease later in life. In contrast, current evidence suggests that most cardiovascular abnormalities in MIS-C improve over time, although long-term longitudinal data remain limited. This review synthesizes current evidence on the pathophysiology, imaging strategies, risk assessment, and long-term cardiovascular outcomes in KD and MIS-C. A multimodal imaging approach combined with individualized risk stratification is essential for improving prognosis and guiding lifelong surveillance in affected patients.

We present the case of a man in his late 50s who was admitted with an acute ischaemic stroke and subsequently developed rapidly progressive encephalopathy, multiple bilateral infarcts and intracranial haemorrhages. Despite initial suspicion of reversible cerebral vasoconstriction syndrome or posterior reversible encephalopathy syndrome, repeated neuroimaging, including high-resolution black-blood MRI, demonstrated vessel wall enhancement consistent with vasculitis. Extensive work-up excluded systemic vasculitis, infection, malignancy and cardioembolic sources. The clinical and radiological findings met the diagnostic criteria for probable primary angiitis of the central nervous system (PACNS). Definite diagnosis of PACNS requires histopathological confirmation by biopsy, which was not performed. Immunosuppressive therapy with high-dose corticosteroids and azathioprine was initiated, leading to a gradual improvement of the encephalopathic state although severe neurological deficits persisted. This case highlights the diagnostic challenges associated with PACNS and underscores the importance of repeated reassessment in patients with unexplained altered consciousness.

To investigate the impact of intravenous immunoglobulin (IVIG) timing on coronary artery outcomes and treatment response in children with Kawasaki disease (KD). This retrospective study analyzed 2,988 pediatric patients with Kawasaki disease (KD) who received initial IVIG treatment within 3-7 days of fever onset. Patients were categorized by IVIG initiation time: Group A (day 3 of fever, n = 134), Group B (day 4 of fever, n = 465), Group C (day 5 of fever, n = 1101), Group D (day 6 of fever, n = 833), and Group E (day 7 of fever, n = 455). We compared baseline characteristics, IVIG resistance, and convalescent-phase coronary artery lesions (CALs). Multivariate logistic regression analyzed the association between early initiation (days 3-4 of fever) of initial IVIG therapy and IVIG resistance or convalescent-phase CALs. Propensity score matching (PSM) was used to match the early (days 3-4 of fever, n = 590) and conventional (days 5-7 of fever, n = 590) treatment groups. Baseline data during the acute phase revealed that with delayed initiation of initial IVIG treatment, serum albumin (ALB), aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels in children with KD showed a significant overall decreasing trend (P < 0.001), while platelet (PLT) counts and the incidence of acute-phase CALs showed a significant overall increase (P < 0.001). However, no significant difference was found in convalescent-phase CALs incidence among groups. IVIG resistance was more common among children who required early treatment (P < 0.001). PSM analysis showed no significant difference in acute or convalescent-phase CALs between the early and conventional groups. In the multivariate logistic regression analysis after adjusting for confounders, early IVIG initiation was not significantly associated with convalescent-phase CALs but was associated with a significantly increased risk of IVIG resistance (OR: 1.74; 95% CI: 1.32-2.29; P < 0.001).Conclusion: In this single-center retrospective study, initiating initial IVIG therapy within 3-7 days of fever onset showed no significant impact on the incidence of convalescent-phase CALs at different treatment timings. However, IVIG resistance was more common among children who required early treatment (days 3-4 of fever). Due to potential residual confounding by indication, these findings represent a non-causal association that may reflect differences in underlying disease severity and clinical presentation. Future prospective, multicenter, randomized controlled trials are needed to further clarify the optimal timing of initial IVIG therapy in KD patients, thereby providing evidence for developing individualized treatment strategies and improving long-term prognosis.

Data on neuro-Behçet's disease in North Africa remain limited and fragmented. We conducted a systematic review and meta-analysis to estimate the frequency of neurologic involvement in North African patients with Behçet's disease and to summarize the main clinical, cerebrospinal fluid, and imaging characteristics. PubMed, Scopus, and Web of Science databases were searched for studies reporting neuro-Behçet's disease in North Africa. Proportions were pooled using a random-effects meta-analysis model to estimate overall pooled proportions (OPP). Heterogeneity was assessed using I[2] statistics. The review protocol was registered in PROSPERO (CRD420261303759). Thirteen studies were included. Among 4262 patients with Behçet's disease, the pooled proportion of Neuro-Behçet's disease was 18.54% (95% CI 13.93% - 24.25%). The overall pooled proportions of parenchymal Neuro-Behçet's disease was 69.19% of cases (95% CI 58.79% - 77.96%). The main neurological symptoms were pyramidal signs (OPP 68.02%; 95% CI 35.27% - 89.25%), headaches (OPP 44.24%; 95% CI 23.16% - 67.63%), dysarthria/speech impairment (OPP 26.72%; 95% CI 13.49% - 46.02%), and cerebellar signs (OPP 23.60%; 95% CI 11.69% - 41.89%). Non-parenchymal involvement was mainly vascular, particularly cerebral venous thrombosis (OPP 25.92%; 95% CI 17.29% - 36.93%). Peripheral neuropathy was also reported (OPP 2.78%; 95% CI 1.22% - 6.24%). Neurological involvement in Behçet's disease is frequent in North Africa with a predominance of parenchymal forms. This first comprehensive meta-analysis benchmarks North African Neuro-Behçet's disease against global patterns and supports integrated clinical diagnosis over reliance on imaging alone.

Distinguishing autoimmune glomerulonephritis from hereditary nephropathy is critical to avoid unnecessary immunosuppression and ensure appropriate long-term renal monitoring. A 31-year-old Hispanic female patient with a complex medical history, including proteinase 3 (PR3)-positive granulomatosis with polyangiitis (GPA), rheumatoid arthritis, Raynaud phenomenon, seizure disorder, autism spectrum disorder, bipolar disorder with psychotic features, and cochlear implantation in childhood, was found to have genetically confirmed Alport syndrome through Natera testing (Natera, Inc., Austin, Texas, United States). Despite preserved renal function (estimated glomerular filtration rate (eGFR) 107 mL/minute/1.73m²), urinalysis demonstrated persistent proteinuria. Her history of early-onset hearing loss requiring cochlear implantation raised suspicion for hereditary nephropathy. Elevated anti-PR3 antibodies were present without clinical evidence of active vasculitis. The coexistence of autoimmune disease and hereditary collagen IV nephropathy complicated the attribution of urinary abnormalities. This case highlights the importance of genetic evaluation in patients with multisystem disease and urinary abnormalities, particularly when clinical features such as early-onset sensorineural hearing loss are present. Recognition of Alport syndrome in patients with coexisting autoimmune vasculitis has significant implications for renal surveillance, immunosuppressive stewardship, and multidisciplinary management.

VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome has protean manifestations, with cutaneous findings being present in the majority of affected individuals. This review covers various signs and findings for which VEXAS syndrome should be considered, with emphasis on dermatologic and dermatopathologic clues.

Precision management of ocular complications in systemic autoimmune diseases, such as Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), Behçet's disease, and thyroid-associated ophthalmopathy (TAO), requires integration of immune mechanisms with clinical translation. These disorders manifest as dry eye, uveitis, retinal vasculitis, optic neuritis, and orbital fibroinflammatory disease, posing a substantial risk of irreversible visual loss. Recent studies highlight the T-helper 17 (Th17)/interleukin-17 (IL-17) axis, tumor necrosis factor-alpha (TNF-α), complement component 5a (C5a), and TAO-related immunostromal remodeling as pivotal inflammatory drivers. Specifically, TAO is increasingly recognized as an immunostromal disorder in which fibroblast-immune crosstalk connects thyrotropin receptor antibody/insulin-like growth factor-1 receptor signaling with orbital edema, adipogenesis, and fibrosis. Disease-specific pathways, including anti-Sjögren's-syndrome-related antigen A (anti-SSA)-mediated lacrimal damage and the human leukocyte antigen B27 (HLA-B27) gut-eye axis, further enrich etiological insights. Diagnostically, aqueous IL-17, C-X-C motif chemokine ligand 10 (CXCL10), tear biomarkers, and optical coherence tomography angiography (OCTA) enable more precise phenotyping, whereas invasive ocular-fluid testing requires careful risk-benefit evaluation. Therapeutically, anti-TNF-α monoclonal antibodies, teprotumumab, and avacopan target critical pathways and improve refractory cases, while Janus kinase inhibitors, B-cell-directed combinations, and engineered regulatory T-cell therapies show promise but require rigorous safety and translational validation. This review discusses major advances in immunopathogenesis, diagnostics, and therapeutics, comparing disease commonalities and distinctions to propose a framework for precision management. It envisions multi-omics phenotyping, artificial intelligence-assisted diagnostics, and pathway-directed immunomodulation to reduce blindness and enhance quality of life, bridging rheumatology and ophthalmology.