Performance of lupus classification criteria in monogenic lupus: a national multicenter cohort study

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ID: 330899
2026
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Abstract
Abstract Objectives With advances in genetic testing, monogenic lupus is increasingly recognized, yet disease-specific classification criteria are lacking. We compared all three classification criteria sets in the same monogenic lupus cohort and, for the first time, evaluated their performance across distinct genetic pathways. Methods This national multicenter study included 88 genetically confirmed monogenic lupus patients from 22 pediatric rheumatology centers across Türkiye. Fulfilment of the ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria and pathway-based differences in classification performance were assessed. Results Overall, 74% (n = 65) fulfilled ACR-1997, 70.5% (n = 62) SLICC-2012 and EULAR/ACR-2019 criteria, and 62.5% (n = 55) met all three, whereas 17% (n = 15) met none. Agreement was substantial (κ = 0.63–0.73), with no significant differences between criteria (p > 0.05). Classification performance differed significantly across genetic pathways (p < 0.05), with the highest fulfilment performances in complement deficiencies and the lowest in nucleic acid metabolism and type I interferon pathway defects. Most patients who did not fulfill any classification criteria had nucleic acid metabolism and clearance defects (13/15, 86.7%). Domain-level analyses showed that classification success was mainly driven by mucocutaneous, immunologic, and renal domains. Complement and immune-regulation defects showed a more classical lupus phenotype, with most fulfilling all three criteria (78.6%, 22/28 and 77.8%, 14/18), whereas the proportion was lower in type I interferon pathway and nucleic acid metabolism and clearance defects (33.3%, 2/6 and 47.2%, 17/36). No classification criteria was superior within genetic pathways. Conclusions One-third of genetically confirmed monogenic lupus patients remained unclassified, particularly those with interferon-related and nucleic acid metabolism defects, highlighting pathway-dependent limitations of current classification criteria.
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Authors Nergis Akay, Sezgin Şahin, Sıla Atamyıldız Uçar, Aybuke Alisan, Eray Tunce, Eda Kayhan, Yasemin Uğur Es, Ayşenur Alkaya, Büşra Başer Taşkın, Dilara Ünal, Lütfiye Koru, Hatice Kubra Zora, Aslıhan Uzun, Hatice Melisa Kaçmaz, Halil Kazanasmaz, Şengül Çağlayan, Seher Şener, Büşra Acun, Gülcan Özomay Baykal, Yasemin Demir Yiğit, Süleyman Ekrem Albayrak, Burcu Bozkaya Yücel, Orhan Nurlu, Mehmet Yıldız, Amra Adroviç, Kenan Barut, Sevcan Azime Bakkaloglu, Metin Kaya Gürgöze, Ayşe Balat, Fatih Haşlak, Balahan Bora Makay, Sara Şebnem Kılıç, Mukaddes Kalyoncu, Oya Köker, Semanur Özdel, Nuray Aktay Ayaz, Seza Özen, Banu Çelikel Acar, Sümeyra Özdemir Çiçek, Sibel Balcı, Ayşenur Paç Kısaarslan, Rabia Miray Kışla Ekinci, Betül Sözeri, Özgür Kasapçopur
Journal Lara D. Veeken
Year 2026
DOI
10.1093/rheumatology/keag532
URL
https://doi.org/10.1093/rheumatology/keag532
Keywords Keywords not found

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