Retrospective study: clinical analysis of Kawasaki disease complicated with macrophage activation syndrome. [PDF]
Bie W, Guan Y, Du Q, Li L, Yu P.
europepmc +1 more source
Objective Still disease (SD) is an autoinflammatory disorder characterized by remarkably high interleukin‐18 (IL‐18) levels. Increasing evidence suggests that adaptive immunity also contributes to its pathogenesis, particularly in refractory courses. Macrophage activation syndrome (MAS), one of SD's most severe complications, is associated with further
Greta Rogani +17 more
wiley +1 more source
Breaking the storm: canakinumab as salvage therapy for refractory macrophage activation syndrome in pediatric systemic lupus erythematosus-a case report. [PDF]
Selvarajan T +4 more
europepmc +1 more source
Objective Information is limited on the natural history and current treatment patterns in macrophage activation syndrome (MAS), a life‐threatening hyperinflammatory syndrome complicating Still's disease (systemic juvenile idiopathic arthritis [sJIA] and adult‐onset Still's disease [AOSD]).
Alexei Grom +21 more
wiley +1 more source
Human Monocytic Models Reveal Genotype‐Dependent Inflammatory Programs in VEXAS Syndrome
Objective VEXAS syndrome is a severe X‐linked autoinflammatory disorder caused by somatic mutations in ubiquitin‐like modifier activating enzyme 1 (UBA1), with clinical outcomes that vary by UBA1 genotype. We aimed to elucidate genotype‐specific inflammatory programs and identify potential therapeutic targets.
Kana Higashitani +10 more
wiley +1 more source
Breaking the Storm: Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome as the First Manifestation of Systemic Lupus Erythematosus/Mixed Connective Tissue Disease in an Adolescent Successfully Treated With Anakinra. [PDF]
Alhasan R +5 more
europepmc +1 more source
Machine learning for predicting macrophage activation syndrome in adult patients with Still's disease. [PDF]
Zheng Y +6 more
europepmc +1 more source
Graphical Abstract Objective This study aimed to characterize whole blood transcriptomic profiles in adults with active Sjögren's disease (SjD) treated with anti‐CD20 or B lymphocyte stimulator/BAFF inhibition, identify markers of clinical response, and define inflammatory pathways linked to nonresponse. Methods Whole blood RNA sequencing was performed
Aimen Ibrahim +9 more
wiley +1 more source
Navigating adverse immunostimulation: A practical guide for clinical researchers
Problem Setting As drug development moves towards more complex products, early clinical development programmes are increasingly hampered by unwanted and/or unexpected activation of the immune system (adverse immune stimulation, AIS). Solution At the Centre for Human Drug Research, we have introduced standardized procedures to make AIS manageable, while
Juliette A. van den Noort +3 more
wiley +1 more source
Macrophage activation syndrome in a 13-month-old child with systemic juvenile idiopathic arthritis mimicking sepsis: a diagnostic challenge and case report. [PDF]
Subedi P +4 more
europepmc +1 more source

