ObjectiveSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has been associated with neurological complications beyond respiratory involvement. Although children usually experience mild systemic disease, immune-mediated central nervous system (CNS) disorders have emerged as an important cause of morbidity. Neuroimaging plays a central role in detecting these complications and supporting diagnostic decisions. This study aims to describe neuroimaging patterns in children who developed immune-mediated CNS disorders following SARS-CoV-2 infection.Materials and methodsWe retrospectively reviewed pediatric patients aged 0-18 years with laboratory-confirmed SARS-CoV-2 infection followed at a tertiary pediatric neurology center. Among post-COVID admissions, four patients with severe neurological involvement and characteristic neuroimaging abnormalities were identified. Clinical features, laboratory findings, cerebrospinal fluid analyses, electroencephalography results, and multimodal neuroimaging studies were evaluated.ResultsFour previously healthy male children developed immune-mediated neurological disorders associated with SARS-CoV-2 infection, including febrile infection-related epilepsy syndrome, acute disseminated encephalomyelitis, central nervous system vasculitis, and anti-N-methyl-D-aspartate receptor encephalitis. Brain MRI demonstrated disorder-specific patterns such as cortical diffusion restriction, multifocal demyelinating lesions, inflammatory vasculitic changes, and progressive parenchymal injury. In one patient, serial imaging showed progression from acute inflammation to chronic structural damage. All patients received immunomodulatory therapy, with variable neurological outcomes.ConclusionSARS-CoV-2 infection may trigger immune-mediated CNS disorders in children with distinct neuroimaging features. Recognition of these patterns is essential for early diagnosis, treatment guidance, and prognostic assessment. Neuroimaging also provides insight into the evolution of post-COVID-19 neuroinflammatory injury.

Dapsone is widely used for inflammatory dermatoses and vasculitides but may cause severe systemic hypersensitivity reactions. Diagnosis can be challenging when clinical features overlap with underlying inflammatory disease and when eosinophilia is absent. We report a case of a young man with biopsy-confirmed immunoglobulin A vasculitis (Henoch-Schönlein purpura) who developed facial and neck edema, a diffuse morbilliform eruption, and hepatocellular liver injury approximately four weeks after initiation and dose escalation of dapsone. Laboratory evaluation demonstrated elevated aminotransferases with normal creatinine and absent eosinophilia, along with anemia and reticulocytosis suggestive of concurrent dapsone-related hematologic toxicity. The patient was receiving ongoing cyclosporine and had recent high-dose corticosteroid exposure at the time of presentation. Dapsone was discontinued and systemic corticosteroids were administered with rapid clinical improvement. This case highlights diagnostic pitfalls in recognizing severe drug reactions in immunosuppressed patients and emphasizes the importance of integrating medication timelines, organ involvement, and immunosuppression status when evaluating suspected drug reactions.

Giant cell arteritis-associated mesenteric vasculitis is a rare manifestation and often carries significant mortality and morbidity. We present a case of GCA with mesenteric involvement that was at first diagnostically challenging, contributed to by a history of functional illness; however, it achieved successful treatment without ischemic complications.

Branched thoracic endovascular aneurysm repair has become an increasingly common intervention since the advent of custom made and now off the shelf devices. This report describes the use of a sequential double deployment of the Gore TAG Thoracic Branch Endoprosthesis (TBE; WL Gore and Associates, Flagstaff, AZ, USA) to exclude a left common carotid artery aneurysm (LCCAA) due to large vessel vasculitis (LVV).

BACKGROUND IgA-associated vasculitis (IgAV), although relatively uncommon in adults, is frequently associated with increased vasculitis severity. Sudden abdominal pain and bloody diarrhea are among the usual manifestations of IgAV in children. In older patients, clinical manifestations of the disease may be typical, but advanced age and comorbidities can make the final diagnosis unexpected. CASE REPORT A 67-year-old man with multiple underlying medical conditions, including diabetes mellitus and arterial hypertension, was admitted with acute abdominal pain. Baseline evaluation revealed venous ischemic necrosis of the ileum and concurrent acute kidney injury. Given the patient's age and diabetic status, an atherogenic etiology was initially considered. Further diagnostic investigation, including renal biopsy, identified features consistent with IgAV. The patient's clinical course was complicated by simultaneous gastrointestinal and renal involvement, highlighting the systemic nature of the vasculitis. CONCLUSIONS Although IgAV is predominantly a pediatric condition, its presentation in older adults is rare and often atypical, commonly mimicking other vascular or ischemic disorders; this pattern may delay diagnosis and appropriate treatment. The present case emphasizes the need to maintain awareness of IgAV in older patients who present with acute abdominal symptoms, particularly when renal dysfunction is present. Advanced age and diabetic status are associated with increased severity and worse renal outcomes, underscoring the need for thorough evaluation and close monitoring. This report contributes to the limited literature on IgAV in older patients; it illustrates the diagnostic and therapeutic challenges in this population.

Intravenous immunoglobulin (IVIg) is the standard of care for the treatment of Kawasaki disease (KD) and should be administered within 10 days of the onset of fever. Management guidelines for children with KD who defervesce spontaneously are not clear. In this study, we analysed patients with KD diagnosed between 1994 and 2024 at our centre who had defervesced spontaneously, had normal acute-phase reactants, and underwent echocardiographic examination, and in whom IVIg had not been administered. We reviewed the records of patients with KD from January 1994 - December 2024. The diagnosis of KD was based on standard guidelines. Patients with KD were said to be in spontaneous defervescence when they remained afebrile for ≥ 48 h, had normal acute-phase reactants [C-reactive protein (CRP), Erythrocyte sedimentation rate (ESR)] and no coronary artery abnormalities (CAAs) on echocardiography at presentation, and when IVIg was not administered. Patients with spontaneous defervescence were subdivided into (i) early defervescence (Ed-KD), if the interval between onset of symptoms and defervescence was < 10 days, and (ii) late defervescence (Ld-KD), if the duration between onset of symptoms and defervescence was ≥ 10 days, respectively. Details of the clinical profile, laboratory investigations, and echocardiography findings were obtained from the records. Of the 1499 patients with KD enrolled during the study period, 115 patients (7.7%; 86 boys) defervesced spontaneously. The median age at disease onset was 6 years (mean, 5.6 years; range, 0.8-15 years). The median duration of fever, defined as the total duration of the febrile episode before spontaneous defervescence, was 5 days (range, 1-21 days). The median interval between illness onset (defined as fever onset) and diagnosis of KD was 15 days (range, 4-40 days), indicating that diagnosis was often made after fever had already subsided. The most common clinical feature was periungual desquamation, followed by rash, oral-mucosal changes, cervical lymphadenopathy, and conjunctival injection. Incomplete presentation was noted in 73.9% (n = 85/115) of patients. No patient has developed CAAs or other cardiac sequelae over a median follow-up of 9 months (range 2 months-156 months). The cumulative follow-up for the cohort was 235 patient-years. The 'low-risk' subgroup of patients with KD who defervesce spontaneously, and have normal acute phase reactants with no CAAs at presentation, have good clinical and coronary outcomes.

Programmed cell death (PCD) is a genetically regulated, orderly cell death process essential for tissue homeostasis. Contemporary PCD comprises a broad spectrum, including apoptosis, pyroptosis, ferroptosis, necroptosis, autophagy-dependent cell death, NETosis, parthanatos, and entotic cell death. Kawasaki disease (KD) is an acute pediatric systemic vasculitis and a leading cause of acquired childhood heart disease, with coronary artery lesions as the most severe complication. Mounting evidence confirms that PCD is tightly associated with KD-related vascular endothelial injury and inflammatory amplification. Among all PCD subtypes, apoptosis, pyroptosis, and ferroptosis have the most sufficient and direct evidence in KD pathogenesis, mediating vascular wall damage and inflammatory imbalance via distinct molecular pathways. This review focuses on these three well-documented PCD forms (with a brief overview of other PCD subtypes and their potential KD relevance), systematically elaborates their crosstalk with KD pathogenesis, summarizes current research progress, and proposes targeted therapeutic strategies for KD.

The integration of coronary sinus flow (CSF) and myocardial deformation imaging represents an emerging non-invasive approach for detecting subclinical cardiac involvement in systemic diseases. In their recent study, Güleşir et al. provide evidence of reduced CSF and impaired biventricular strain in patients with Behçet's disease (BD), suggesting a potential link between coronary microvascular dysfunction and myocardial mechanical alterations. While this interpretation is biologically plausible in the context of systemic vasculitis, alternative mechanisms may contribute to the observed findings. In particular, extrinsic mechanical factors related to thoracic shape that affect cardiac geometry and venous drainage may simultaneously influence both CSF and myocardial strain parameters. Insights from conditions such as pectus excavatum, where chest wall deformity induces apparent reductions in myocardial strain despite preserved intrinsic function, highlight the potential role of mechanical constraint as a confounding factor. This commentary discusses the interplay between coronary flow, myocardial deformation, and cardiac mechanics, emphasizing the need for a more nuanced interpretation of echocardiographic findings. A comprehensive approach integrating structural, hemodynamic, and microvascular determinants may be essential to accurately characterize subclinical cardiac involvement in BD and other systemic conditions.

Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening hyperinflammatory conditions with heterogeneous triggers and overlapping clinical phenotypes. Diagnostic uncertainty is particularly challenging in resource-limited settings, where advanced molecular and immunophenotypic investigations are not readily available. A 31-year-old woman presented with recurrent febrile episodes, trilineage cytopenias, hyperferritinemia (peak 16,970 ng/mL [reference: < 200 ng/mL]), hypofibrinogenemia, and progressive necrotic panniculitis plaques involving both lower extremities. Histopathological evaluation revealed lobular panniculitis with adipocyte necrosis and absence of vasculitis. While these findings raised strong concern for subcutaneous panniculitis-like T-cell lymphoma (SPTCL), definitive diagnosis was precluded by the absence of immunophenotyping, T-cell receptor clonality studies, and HAVCR2 mutation testing-all unavailable in our setting. The HScore was 216 (> 99% probability of HLH/MAS). A striking feature was the persistent dissociation between markedly elevated ferritin and low C-reactive protein (CRP) values (ratio < 0.002), consistent with a non-IL-6-dominant, interferon-γ-driven hyperinflammatory state. Based on this phenotype, a sequential treatment strategy combining therapeutic plasma exchange (TPE), intravenous immunoglobulin (IVIG), and cyclosporine A (CyA) was implemented without cytotoxic chemotherapy. The patient demonstrated significant clinical and biochemical improvement, with resolution of cytopenias and progressive decline in ferritin. This case highlights the diagnostic overlap between autoimmune panniculitis, SPTCL, and HLH/MAS, and underscores the importance of phenotype-guided management in complex hyperinflammatory syndromes. It further illustrates that effective treatment can be achieved without cytotoxic therapy in selected patients, even in the absence of definitive molecular diagnosis.