Immune regulatory mechanisms and potential microbiota-associated targets in Kawasaki disease: an integrative multi-omics and network pharmacology study.
Kawasaki disease (KD) is a systemic vasculitis in children primarily affecting the coronary arteries, and studies suggest that the gut microbiota may be involved in KD pathogenesis, inflammatory responses, and immune regulation. This study employed an integrative multi-omics strategy to systematical…
Idiopathic IgG4-negative perivascular fibrosis with extensive thoracic aortic, coronary artery, and supra-aortic branch involvement: A case report.
Perivascular fibrosis is an uncommon fibroinflammatory disorder characterized by circumferential soft-tissue thickening surrounding the aorta and its major branches. Involvement of the thoracic region and supra-aortic branches is less common, potentially leading to imaging studies that can resemble…
From presumed infection to systemic vasculitis: de novo granulomatosis with polyangiitis in pregnancy - a case report.
Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated vasculitis. De novo presentation during pregnancy is exceptionally rare and may mimic severe infection, delaying diagnosis. This report concerns a case of new-onset GPA in a 26-year-old primigravid woman presenting in the second trimes…
ADA2 genotype and enzyme activity may predict vasculitic or hematologic DADA2 phenotype.
Deficiency of adenosine deaminase 2 (DADA2) is an autoinflammatory disease with diverse phenotypes. We describe the genetics, phenotypes, and treatment of n = 48 DADA2 patients from Germany, Austria, and Switzerland. We report a high incidence of hematological (83%) and immunological features (85%),…
Quantitative iodine-mapping of photon-counting CT detects large-vessel vasculitis: A case report.
Diagnosis of large-vessel vasculitis can be challenging because clinical symptoms and early imaging findings are often nonspecific. A 69-year-old woman presented to the emergency department with severe headache and markedly elevated inflammatory markers. Initial brain CT, brain MRI, and ultrasound e…
Association of FHR5 With Disease Severity in ANCA-Associated Vasculitis.
Alternative complement activation is pivotal in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) pathogenesis. Growing evidence highlights an important role of complement factor H (FH) in AAV. FH-related protein 5 (FHR5) functions as a competitive antagonist of FH. This study a…
Isolated nasal septal involvement in granulomatosis with polyangiitis: Radiologic features and literature review.
Granulomatosis with polyangiitis is a necrotizing granulomatous vasculitis that predominantly involves the upper and lower respiratory tracts and the kidneys. Although sinonasal manifestations are common, they are often nonspecific and can mimic benign inflammatory conditions, leading to delayed dia…