Complement Activation Linked to Type II Interferon Signaling in Still Disease
Objective Still disease (SD) is an autoinflammatory syndrome characterized by innate immune dysregulation. Although complement can drive inflammation, its involvement in SD remains to be defined. Thus, we aimed to assess complement activation in SD. Methods Complement was assessed using transcriptomic, proteomic, and in vitro approaches. RNA sequencing
Freya M. C. H. Huijsmans +115 more
wiley +1 more source
Opinion: Why paediatric rheumatologists need to understand inborn errors of immunity. [PDF]
Abinun M, Owens S.
europepmc +1 more source
Objective Tissue‐specific biomarkers that are reliable and associated with clinical manifestations of idiopathic inflammatory myopathy (IIM) are lacking. The blood circulation in individuals serves as a central conduit, allowing communication between tissues and facilitating clearance and recycling of tissue‐derived proteins.
Yue‐Bei Luo +23 more
wiley +1 more source
Ichthyosis prematurity syndrome mimicking hyper-IgE syndrome due to a novel SLC27A4 variant. [PDF]
Akal C +7 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
Delayed Diagnosis of Germline <i>GATA2</i> Deficiency Following Severe Adolescent Iliocaval Thrombosis and Decade-Long Monocytopenia: Diagnostic and Testing Pitfalls. [PDF]
Wahab A, Zahweh M, Stone M, Quigley JG.
europepmc +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
Commentary: Anti-endothelial cell antibodies in pathogenesis of vasculitis. [PDF]
Xie J, Shi L, Wang L, Dai J.
europepmc +1 more source
Expert Perspectives: Toward Treat‐to‐Target Management in Adult Idiopathic Inflammatory Myopathies
Treat‐to‐target has transformed the management of several rheumatic diseases through predefined therapeutic goals, structured disease assessment, and timely treatment adjustment. Despite major advances in the understanding and treatment of idiopathic inflammatory myopathies (IIM), a formal treat‐to‐target framework for adult IIM has not yet been ...
Shiri Keret +2 more
wiley +1 more source
How Nutrient Deficiencies Impact the Oral Mucosa and How to Manage Them: A Narrative Review. [PDF]
Starzyńska P +3 more
europepmc +1 more source

