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IFN-γ licenses normal and pathogenic ALPK1/TIFA pathway in human monocytes.
iScience
January 2025
CIRI, Centre International de Recherche en Infectiologie, Inserm U1111, Université Claude Bernard Lyon 1, CNRS, UMR5308, ENS de Lyon, University Lyon, F-69007 Lyon, France.
Alpha-kinase 1 (ALPK1) is an immune receptor sensing the bacterial nucleotide sugar ADP-heptose. ALPK1 phosphorylates TIFA leading to its oligomerization and downstream NF-κB activation. Specific mutations in are associated with an autoinflammatory syndrome termed ROSAH and with spiradenoma (skin cancers with sweat gland differentiation).
View Article and Find Full Text PDFUnderstanding the Intricate Pathophysiology of Psoriasis and Related Skin Disorders.
Int J Mol Sci
January 2025
Department of Dermatology, Faculty of Medicine, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania.
Psoriasis is a chronic inflammatory condition that is polygenic and multisystemic, impacting approximately 2-3% of the global population. The onset of this disease is influenced by an intricate interplay of genetic and environmental factors, predisposing individuals to the psoriasis phenotype. The complex pathogenesis of psoriasis contains certain key aspects found in other autoinflammatory and autoimmune dermatological diseases.
View Article and Find Full Text PDFClinical and Imaging Characteristics of Posterior Scleritis in Children.
Ocul Immunol Inflamm
January 2025
Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.
Background: Posterior scleritis (PS) is a rare phenotype of scleritis. Comprehensive epidemiological studies on PS in children are limited. We aimed to report on its clinical and imaging features in one of the largest pediatric series to date.
View Article and Find Full Text PDFAssociated diseases and their effects on disease course in patients with chronic non-bacterial osteomyelitis: retrospective experience from a single center.
Clin Rheumatol
January 2025
Division of Pediatric Rheumatology and Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.
Old and New Biomarkers in Idiopathic Recurrent Acute Pericarditis (IRAP): Prognosis and Outcomes.
Curr Cardiol Rep
January 2025
Division of Internal Medicine, Fatebenefratelli Hospital, ASST Fatebenefratelli Sacco, University of Milan, Piazzale Principessa Clotilde, 3, Milan, 20121, Italy.
Purpose Of Review: To outline the latest discoveries regarding the utility and reliability of serum biomarkers in idiopathic recurrent acute pericarditis (IRAP), considering recent findings on its pathogenesis. The study highlights the predictive role of these biomarkers in potential short- (cardiac tamponade, recurrences) and long-term complications (constrictive pericarditis, death).
Recent Findings: The pathogenesis of pericarditis has been better defined in recent years, focusing on the autoinflammatory pathway.
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