Both granulomatous polyangiitis (GPA) and Crohn's disease (CD) can cause orbital inflammation though rarely coincide and can often be differentiated by presenting features and histological findings. Here, we report the clinical and therapeutic course of a 14-year-old White male with binocular diplopia caused by orbital myositis. Imaging and biopsy obtained at presentation revealed necrosis and necrotizing granulomatous vasculitis suspicious for GPA.
View Article and Find Full Text PDFIncreasing molecular knowledge of autoinflammatory and autoimmune disorders has enabled more targeted treatment of these conditions. Treatment of inflammasomopathies is often aimed at interleukin-1 (IL-1) blockade, with potential use of other inhibitors targeting cytokines such as IL-18 and IL-6. Interferonopathies and some disorders with overlap features of autoimmunity and autoinflammation may improve with Janus kinase inhibition.
View Article and Find Full Text PDFThe impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID Working Group aimed to investigate the impact of the COVID-19 pandemic on the number of pediatric patients evaluated for recurrent fevers and autoinflammatory diseases in North America. The absolute number of new outpatient visits and the proportion of these visits attributed to recurrent fever diagnoses during the pre-pandemic period (1 March 2019-29 February 2020) and the first year of the COVID-19 pandemic (1 March 2020-28 February 2021) were examined.
View Article and Find Full Text PDFBackground: We studied the impact of the multicomponent interventions on body weight and cardiometabolic risk factors in overweight individuals working in corporate worksites.
Methods: Overweight (BMI ≥ 23 kg/m) subjects were recruited from four randomised worksites [two active intervention (, recruited, 180, completed 156) and two control (, recruited 130, completed 111)]. Intensive intervention was given at intervention worksite.
Objective: The nuclear oncoprotein DEK is an autoantigen associated with juvenile idiopathic arthritis (JIA), especially the oligoarticular subtype. DEK is a secreted chemotactic factor. Abundant levels of DEK and DEK autoantibodies are found in inflamed synovium in JIA.
View Article and Find Full Text PDFNovel therapeutics are required for improving the management of chronic inflammatory diseases. Aptamers are single-stranded RNA or DNA molecules that have recently shown utility in a clinical setting, as they can specifically neutralize biomedically relevant proteins, particularly cell surface and extracellular proteins. The nuclear chromatin protein DEK is a secreted chemoattractant that is abundant in the synovia of patients with juvenile idiopathic arthritis (JIA).
View Article and Find Full Text PDFObjective: Systemic lupus erythematosus (SLE) is characterized by increased cardiovascular risk in adult-onset and childhood-onset SLE. Type I interferons (IFNs) appear to play a prominent role in premature vascular damage in adult-onset SLE, at least in part, by inducing impairments in the phenotype and function of endothelial progenitor cells (EPCs), thereby hampering vascular repair. It is not clear whether EPC dysfunction is present in childhood-onset SLE in association with a type I IFN signature.
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