EULAR 2026 Editors' Top Picks

State of the Art REVIEW

108 Niewold TB, Hua J, Lehman TJ, Harley JB, Crow MK. High serum IFN-alpha activity is a heritable risk factor for systemic lupus erythematosus. Genes Immun 2007;8:492-502. doi:10.1038/ sj.gene.6364408. 109 Mathian A, Mouries-Martin S, Dorgham K, et al. Ultrasensitive serum interferon-α quantification during SLE remission identifies patients at risk for relapse. Ann Rheum Dis 2019;78:1669-76. doi:10.1136/ annrheumdis-2019-215571. 110 Northcott M, Gearing LJ, Nim HT, et al. Glucocorticoid gene signatures in systemic lupus erythematosus and the effects of type I interferon: a cross-sectional and in-vitro study. Lancet Rheumatol 2021;3:e357- 70. doi:10.1016/S2665-9913(21)00006-0. 111 Bruce IN, van Vollenhoven RF, Morand EF, et al. Sustained glucocorticoid tapering in the phase 3 trials of anifrolumab: a post hoc analysis of the TULIP-1 and TULIP-2 trials. Rheumatology (Oxford) 2023;62:1526-34. doi:10.1093/rheumatology/keac491. 112 Morand EF, Furie R, Tanaka Y, et al, TULIP-2 Trial Investigators. Trial of anifrolumab in active systemic lupus erythematosus. N Engl J Med 2020;382:211-21. doi:10.1056/NEJMoa1912196. 113 Furie RA, Morand EF, Bruce IN, et al. Type I interferon inhibitor anifrolumab in active systemic lupus erythematosus (TULIP-1): a randomised, controlled, phase 3 trial. Lancet Rheumatol 2019;1:e208-19. doi:10.1016/S2665-9913(19)30076- 1. 114 Psarras A, Wittmann M, Vital EM. Emerging concepts of type I interferons in SLE pathogenesis and therapy. Nat Rev Rheumatol 2022;18:575-90. doi:10.1038/s41584-022-00826-z. 115 Psarras A, Alase A, Antanaviciute A, et al. Functionally impaired plasmacytoid dendritic cells and non-haematopoietic sources of type I interferon characterize human autoimmunity. Nat Commun 2020;11:6149. doi:10.1038/s41467-020-19918-z. 116 Swiecki M, Colonna M. The multifaceted biology of plasmacytoid dendritic cells. Nat Rev Immunol 2015;15:471-85. doi:10.1038/ nri3865. 117 Chang DM, Lan JL, Lin HY, Luo SF. Dehydroepiandrosterone treatment of women with mild-to-moderate systemic lupus erythematosus: a multicenter randomized, double-blind, placebo-controlled trial. Arthritis Rheum 2002;46:2924-7. doi:10.1002/art.10615. 118 van Vollenhoven RF, Park JL, Genovese MC, West JP, McGuire JL. A double-blind, placebo-controlled, clinical trial of dehydroepiandrosterone in severe systemic lupus erythematosus. Lupus 1999;8:181-7. doi:10.1191/096120399678847588. 119 Petri MA, Mease PJ, Merrill JT, et al. Effects of prasterone on disease activity and symptoms in women with active systemic lupus erythematosus. Arthritis Rheum 2004;50:2858-68. doi:10.1002/ art.20427. 120 Merrill JT, Neuwelt CM, Wallace DJ, et al. Efficacy and safety of rituximab in moderately-to-severely active systemic lupus erythematosus: the randomized, double-blind, phase II/III systemic lupus erythematosus evaluation of rituximab trial. Arthritis Rheum 2010;62:222-33. doi:10.1002/art.27233. 121 Merrill JT, Burgos-Vargas R, et al, Westhovens R. The efficacy and safety of abatacept in patients with non-life-threatening manifestations of systemic lupus erythematosus: results of a twelve- month, multicenter, exploratory, phase IIb, randomized, double- blind, placebo-controlled trial. Arthritis Rheum 2010;62:3077-87. doi:10.1002/art.27601. 122 Wallace DJ, Stohl W, Furie RA, et al. A phase II, randomized, double- blind, placebo-controlled, dose-ranging study of belimumab in patients with active systemic lupus erythematosus. Arthritis Rheum 2009;61:1168-78. doi:10.1002/art.24699. 123 Navarra SV, Guzmán RM, Gallacher AE, et al, BLISS-52 Study Group. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet 2011;377:721-31. doi:10.1016/S0140- 6736(10)61354-2. 124 Furie R, Petri M, Zamani O, et al, BLISS-76 Study Group. A phase III, randomized, placebo-controlled study of belimumab, a monoclonal antibody that inhibits B lymphocyte stimulator, in patients with systemic lupus erythematosus. Arthritis Rheum 2011;63:3918-30. doi:10.1002/art.30613. 125 Urowitz MB, Isenberg DA, Wallace DJ. Safety and efficacy of hCDR1 (Edratide) in patients with active systemic lupus erythematosus: results of phase II study. Lupus Sci Med 2015;2:e000104. doi:10.1136/lupus-2015-000104. 126 Wallace DJ, Kalunian K, Petri MA, et al. Efficacy and safety of epratuzumab in patients with moderate/severe active systemic lupus erythematosus: results from EMBLEM, a phase IIb, randomised, double-blind, placebo-controlled, multicentre study. Ann Rheum Dis 2014;73:183-90. doi:10.1136/annrheumdis-2012-202760. 127 Clowse MEB, Wallace DJ, Furie RA, et al. Efficacy and safety of epratuzumab in moderately to severely active systemic lupus erythematosus: results from the phase 3, randomized, double-blind, placebo-controlled trials, EMBODY™ 1 and EMBODY™ 2. Arthritis Rheumatol 2017;69:362-75. doi:10.1002/art.39856.

84 Burnham TK, Neblett TR, Fine G. The application of the fluorescent antibody technic to the investigation of lupus erythematosus and various dermatoses. J Invest Dermatol 1963;41:451-6. doi:10.1038/ jid.1963.140. 85 Cormane RH. “Bound” globulin in the skin of patients with chronic discoid lupus erythematosus and systemic lupus erythematosus. Lancet 1964;1:534-5. doi:10.1016/S0140-6736(64)92917-4. 86 Bajema IM, Wilhelmus S, Alpers CE, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices. Kidney Int 2018;93:789-96. doi:10.1016/j.kint.2017.11.023. 87 Al-Mayouf SM, Sunker A, Abdwani R, et al. Loss-of-function variant in DNASE1L3 causes a familial form of systemic lupus erythematosus. Nat Genet 2011;43:1186-8. doi:10.1038/ng.975. 88 Hartl J, Serpas L, Wang Y, et al. Autoantibody-mediated impairment of DNASE1L3 activity in sporadic systemic lupus erythematosus. J Exp Med 2021;218:e20201138. doi:10.1084/jem.20201138. 89 Sisirak V, Sally B, D’Agati V, et al. Digestion of chromatin in apoptotic cell microparticles prevents autoimmunity. Cell 2016;166:88-101. doi:10.1016/j.cell.2016.05.034. 90 Gupta S, Kaplan MJ. Bite of the wolf: innate immune responses propagate autoimmunity in lupus. J Clin Invest 2021;131:e144918. doi:10.1172/JCI144918. 91 Denny MF, Yalavarthi S, Zhao W, et al. A distinct subset of proinflammatory neutrophils isolated from patients with systemic lupus erythematosus induces vascular damage and synthesizes type I IFNs. J Immunol 2010;184:3284-97. doi:10.4049/jimmunol.0902199. 92 Carlucci PM, Purmalek MM, Dey AK, et al. Neutrophil subsets and their gene signature associate with vascular inflammation and coronary atherosclerosis in lupus. JCI Insight 2018;3:e99276. doi:10.1172/ jci.insight.99276. 93 Silvestre-Roig C, Braster Q, Wichapong K, et al. Externalized histone H4 orchestrates chronic inflammation by inducing lytic cell death. Nature 2019;569:236-40. doi:10.1038/s41586-019-1167-6. 94 Fernandez-Ruiz R, Belmont HM. The role of anticomplement therapy in lupus nephritis. Transl Res 2022;245:1-17. doi:10.1016/j. trsl.2022.02.001. 95 Botto M, Dell’Agnola C, Bygrave AE, et al. Homozygous C1q deficiency causes glomerulonephritis associated with multiple apoptotic bodies. Nat Genet 1998;19:56-9. doi:10.1038/ng0598-56 96 Garcia-Romo GS, Caielli S, Vega B, et al. Netting neutrophils are major inducers of type I IFN production in pediatric systemic lupus erythematosus. Sci Transl Med 2011;3:73ra20. doi:10.1126/ scitranslmed.3001201. 97 Baumann I, Kolowos W, Voll RE, et al. Impaired uptake of apoptotic cells into tingible body macrophages in germinal centers of patients with systemic lupus erythematosus. Arthritis Rheum 2002;46:191- 201. doi:10.1002/1529-0131(200201)46:1<191::AID- ART10027>3.0.CO;2-K 98 Pisitkun P, Deane JA, Difilippantonio MJ, Tarasenko T, Satterthwaite AB, Bolland S. Autoreactive B cell responses to RNA-related antigens due to TLR7 gene duplication. Science 2006;312:1669-72. doi:10.1126/science.1124978. 99 Jenks SA, Cashman KS, Zumaquero E, et al. Distinct effector B cells induced by unregulated Toll-like receptor 7 contribute to pathogenic responses in systemic lupus erythematosus. Immunity 2018;49:725- 739.e6. doi:10.1016/j.immuni.2018.08.015. 100 Soni C, Perez OA, Voss WN, et al. Plasmacytoid dendritic cells and Type I interferon promote extrafollicular B cell responses to extracellular self-DNA. Immunity 2020;52:1022-1038.e7. doi:10.1016/j.immuni.2020.04.015. 101 Brown GJ, Cañete PF, Wang H, et al. TLR7 gain-of-function genetic variation causes human lupus. Nature 2022;605:349-56. doi:10.1038/s41586-022-04642-z. 102 Lood C, Blanco LP, Purmalek MM, et al. Neutrophil extracellular traps enriched in oxidized mitochondrial DNA are interferogenic and contribute to lupus-like disease. Nat Med 2016;22:146-53. doi:10.1038/nm.4027. 103 Skopelja-Gardner S, An J, Tai J, et al, The early local and systemic Type I interferon responses to ultraviolet B light exposure are cGAS dependent. Sci Rep 2020;13;7908. doi:10.1038/s41598-020-64865-w. 104 An J, Durcan L, Karr RM, et al. Expression of cyclic GMP-AMP synthase in patients with systemic lupus erythematosus. Arthritis Rheumatol 2017;69:800-7. doi:10.1002/art.40002. 105 Kato Y, Park J, Takamatsu H, et al. Apoptosis-derived membrane vesicles drive the cGAS-STING pathway and enhance type I IFN production in systemic lupus erythematosus. Ann Rheum Dis 2018;77:1507-15. doi:10.1136/annrheumdis-2018-212988. 106 Morand EF, Furie R, Tanaka Y, et al, TULIP-2 Trial Investigators. Trial of anifrolumab in active systemic lupus erythematosus. N Engl J Med 2020;382:211-21. doi:10.1056/NEJMoa1912196. 107 Niewold TB. Interferon alpha as a primary pathogenic factor in human lupus. J Interferon Cytokine Res 2011;31:887-92. doi:10.1089/ jir.2011.0071.

the bmj | BMJ 2023;383:073980 | doi: 10.1136/bmj-2022-073980

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