ReviewNature reviews. Rheumatology2025
Emerging concepts and treatments in autoinflammatory interferonopathies and monogenic systemic lupus erythematosus.
Review in Nature reviews. Rheumatology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed.
- The cGAS-STING pathway: mechanistic basis, dual biological functions and translational therapeutic advances.Apoptosis : an international journal on programmed cell death · 2026Review
- Systemic Autoinflammatory Diseases: An Evolving Paradigm of Fevers Beyond Autoimmunity and Infections.Indian pediatrics · 2026Review
- When less is more: loss of IRAK2 signalling promotes inflammation.Nature reviews. Rheumatology · 2026Article
- Type I Interferonopathies: Fifteen Years On, From Concept to Therapeutic Perspectives.Current rheumatology reports · 2026Review
- Spotlight on cGAS-STING: role in disease pathogenesis and therapeutic potential.Molecular biomedicine · 2026Review
- Dual Covalent Targeting of STING Cysteines 292/309 Disrupts Functional Oligomerization and Enables Potent Antagonist Development.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Autoimmune Retinopathy Complicating TLR7-related Monogenic Interferonopathy.Journal of clinical immunology · 2026Article
- Neurodevelopmental comorbidities in juvenile systemic autoimmune and autoinflammatory diseases.Nature reviews. Rheumatology · 2026Review
- COPA syndrome spans multiple organs but is defined by STING in the lung.Nature reviews. Rheumatology · 2026Article
- STING-STAT3-SOX18 Axis Drives EndMT and Epigenetic Reprogramming in SAVI Lung Fibrosis.bioRxiv : the preprint server for biology · 2026Article
- Genetic and phenotypic landscape of monogenic lupus: insights from an international cohort.Lupus science & medicine · 2026Article
- First successful allogeneic hematopoietic stem cell transplantation in STING-associated vasculopathy of infancy-A case report.Molecular therapy. Advances · 2026Article
- Clinical Response to Baricitinib in Monogenic Lupus: Real-World Evidence from a Case Series of Seven Patients.Mediterranean journal of rheumatology · 2026Article
- Molecular mechanisms in rare proteasomopathies.EULAR rheumatology open · 2026Review
- Upregulated ZBP1 Is Associated with B-Cell Dysregulation in Systemic Lupus Erythematosus.Biomedicines · 2026Article
- The emerging clinical relevance of cell-free DNA in lupus: from mechanistic insights to therapeutic opportunities.Frontiers in immunology · 2026Review
- The Gut Microbiota: Emerging Evidence in Autoimmune and Inflammatory Diseases.Research (Washington, D.C.) · 2026Review
- Case Report: Transient immune dysregulation in early infancy mimicking severe systemic autoimmunity with complete spontaneous resolution.Frontiers in immunology · 2026Article
- T cell exhaustion: a two-sided blade in systemic lupus erythematosus, from molecular mechanisms to clinical translation.Frontiers in immunology · 2026Review
- AFG2B gene variants and elevated protein expression in lupus nephritis: new insights into childhood-onset systemic lupus erythematosus.Advances in rheumatology (London, England) · 2025Observational
Corrections and comments
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Authors and funding
3 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Over the past two decades, the number of genetically defined autoinflammatory interferonopathies has steadily increased. Aicardi-Goutières syndrome and proteasome-associated autoinflammatory syndromes (PRAAS, also known as CANDLE) are caused by genetic defects that impair homeostatic intracellular nucleic acid and protein processing respectively. Research into these genetic defects revealed intracellular sensors that activate type I interferon production. In SAVI and COPA syndrome, genetic defects that cause chronic activation of the dinucleotide sensor stimulator of interferon genes (STING) share features of lung inflammation and fibrosis; and selected mutations that amplify interferon-α/β receptor signalling cause central nervous system manifestations resembling Aicardi-Goutières syndrome. Research into the monogenic causes of childhood-onset systemic lupus erythematosus (SLE) demonstrates the pathogenic role of autoantibodies to particle-bound extracellular nucleic acids that distinguishes monogenic SLE from the autoinflammatory interferonopathies. This Review introduces a classification for autoinflammatory interferonopathies and discusses the divergent and shared pathomechanisms of interferon production and signalling in these diseases. Early success with drugs that block type I interferon signalling, new insights into the roles of cytoplasmic DNA or RNA sensors, pathways in type I interferon production and organ-specific pathology of the autoinflammatory interferonopathies and monogenic SLE, reveal novel drug targets that could personalize treatment approaches.
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