Journal of Translational Autoimmunity (Dec 2025)
Dysregulation of innate and adaptive lymphoid immunity may have implications for symptom attribution and predict responses to targeted therapies in neuropsychiatric systemic lupus erythematosus
- Julius Lindblom,
- Guillermo Barturen,
- Lorenzo Beretta,
- Daniel Toro-Domínguez,
- Elena Carnero-Montoro,
- Maria Orietta Borghi,
- Jessica Castillo,
- Ellen Iacobaeus,
- Yvonne Enman,
- Chandra Mohan,
- Marta E. Alarcón-Riquelme,
- Dionysis Nikolopoulos,
- Ioannis Parodis,
- Jacques-Olivier Pers,
- Alain Saraux,
- Valérie Devauchelle-Pensec,
- Sandrine Jousse-Joulin,
- Bernard Lauwerys,
- Julie Ducreux,
- Anne-Lise Maudoux,
- Ana Tavares,
- Isabel Almeida,
- Miguel Angel Gonzalez-Gay Mantecón,
- Ricardo Blanco Alonso,
- Alfonso Corrales Martínez,
- Ignasi Rodríguez-Pintó,
- Gerard Espinosa,
- Rik Lories,
- Nicolas Hunzelmann,
- Doreen Belz,
- Niklas Baerlecken,
- Michael Zauner,
- Michaela Lehner,
- Eduardo Collantes,
- Ma Angeles Aguirre-Zamorano,
- Alejandro Escudero-Contreras,
- Ma Carmen Castro-Villegas,
- Norberto Ortego,
- María Concepción Fernández Roldán,
- Enrique Raya,
- Inmaculada Jiménez Moleón,
- Enrique de Ramon,
- Isabel Díaz Quintero,
- Pier Luigi Meroni,
- Tommaso Schioppo,
- Carolina Artusi,
- Carlo Chizzolini,
- Aleksandra Zuber,
- Donatienne Wynar,
- Attila Balog,
- Magdolna Deák,
- Márta Bocskai,
- Sonja Dulic,
- Gabriella Kádár,
- Falk Hiepe,
- Silvia Thiel,
- Manuel Rodriguez Maresca,
- Antonio López-Berrio,
- Rocío Aguilar-Quesada,
- Héctor Navarro-Linares
Affiliations
- Julius Lindblom
- Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden; Center for Molecular Medicine, Stockholm, Sweden; Corresponding author. Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden.
- Guillermo Barturen
- GENYO, Centre for Genomics and Oncological Research: Pfizer, University of Granada / Andalusian Regional Government, Medical Genomics, Granada, Spain; Department of Genetics, Faculty of Sciences, University of Granada, Granada, Spain
- Lorenzo Beretta
- Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Italy
- Daniel Toro-Domínguez
- GENYO, Centre for Genomics and Oncological Research: Pfizer, University of Granada / Andalusian Regional Government, Medical Genomics, Granada, Spain
- Elena Carnero-Montoro
- GENYO, Centre for Genomics and Oncological Research: Pfizer, University of Granada / Andalusian Regional Government, Medical Genomics, Granada, Spain
- Maria Orietta Borghi
- Department of Clinical Sciences and Community Health, Università Degli Studi di Milano, Milan, Italy; IRCCS, Istituto Auxologico Italiano, Milan, Italy
- Jessica Castillo
- Department of Biomedical Engineering, University of Houston, Houston, TX, USA
- Ellen Iacobaeus
- Neuroimmunology Unit, Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
- Yvonne Enman
- Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden; Center for Molecular Medicine, Stockholm, Sweden
- Chandra Mohan
- Department of Biomedical Engineering, University of Houston, Houston, TX, USA
- Marta E. Alarcón-Riquelme
- GENYO, Centre for Genomics and Oncological Research: Pfizer, University of Granada / Andalusian Regional Government, Medical Genomics, Granada, Spain; Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden
- Dionysis Nikolopoulos
- Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden; Center for Molecular Medicine, Stockholm, Sweden
- Ioannis Parodis
- Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden; Center for Molecular Medicine, Stockholm, Sweden; Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden; Corresponding author. Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, and Karolinska University Hospital, SE-17176, Stockholm, Sweden.
- Jacques-Olivier Pers
- Centre Hospitalier Universitaire de Brest, Hospital de la Cavale Blanche, Brest, France
- Alain Saraux
- Centre Hospitalier Universitaire de Brest, Hospital de la Cavale Blanche, Brest, France
- Valérie Devauchelle-Pensec
- Centre Hospitalier Universitaire de Brest, Hospital de la Cavale Blanche, Brest, France
- Sandrine Jousse-Joulin
- Centre Hospitalier Universitaire de Brest, Hospital de la Cavale Blanche, Brest, France
- Bernard Lauwerys
- Pôle de pathologies rhumatismales systémiques et inflammatoires, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
- Julie Ducreux
- Pôle de pathologies rhumatismales systémiques et inflammatoires, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
- Anne-Lise Maudoux
- Pôle de pathologies rhumatismales systémiques et inflammatoires, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
- Ana Tavares
- Centro Hospitalar do Porto, Portugal
- Isabel Almeida
- Centro Hospitalar do Porto, Portugal
- Miguel Angel Gonzalez-Gay Mantecón
- Servicio Cantabro de Salud, Hospital Universitario Marqués de Valdecilla, Santander, Spain
- Ricardo Blanco Alonso
- Servicio Cantabro de Salud, Hospital Universitario Marqués de Valdecilla, Santander, Spain
- Alfonso Corrales Martínez
- Servicio Cantabro de Salud, Hospital Universitario Marqués de Valdecilla, Santander, Spain
- Ignasi Rodríguez-Pintó
- Department of Autoimmune Diseases, Hospital Clínic, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Catalonia, Spain
- Gerard Espinosa
- Department of Autoimmune Diseases, Hospital Clínic, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Catalonia, Spain
- Rik Lories
- Katholieke Universiteit Leuven, Belgium
- Nicolas Hunzelmann
- Klinikum der Universitaet zu Koeln, Cologne, Germany
- Doreen Belz
- Klinikum der Universitaet zu Koeln, Cologne, Germany
- Niklas Baerlecken
- Medizinische Hochschule Hannover, Germany
- Michael Zauner
- Medical University Vienna, Vienna, Austria
- Michaela Lehner
- Medical University Vienna, Vienna, Austria
- Eduardo Collantes
- Servicio Andaluz de Salud, Hospital Universitario Reina Sofía Córdoba, Spain
- Ma Angeles Aguirre-Zamorano
- Servicio Andaluz de Salud, Hospital Universitario Reina Sofía Córdoba, Spain
- Alejandro Escudero-Contreras
- Servicio Andaluz de Salud, Hospital Universitario Reina Sofía Córdoba, Spain
- Ma Carmen Castro-Villegas
- Servicio Andaluz de Salud, Hospital Universitario Reina Sofía Córdoba, Spain
- Norberto Ortego
- Servicio Andaluz de Salud, Complejo hospitalario Universitario de Granada (Hospital Universitario San Cecilio), Spain
- María Concepción Fernández Roldán
- Servicio Andaluz de Salud, Complejo hospitalario Universitario de Granada (Hospital Universitario San Cecilio), Spain
- Enrique Raya
- Servicio Andaluz de Salud, Complejo hospitalario Universitario de Granada (Hospital Virgen de las Nieves), Spain
- Inmaculada Jiménez Moleón
- Servicio Andaluz de Salud, Complejo hospitalario Universitario de Granada (Hospital Virgen de las Nieves), Spain
- Enrique de Ramon
- Servicio Andaluz de Salud, Hospital Regional Universitario de Málaga, Spain
- Isabel Díaz Quintero
- Servicio Andaluz de Salud, Hospital Regional Universitario de Málaga, Spain
- Pier Luigi Meroni
- Università degli studi di Milano, Milan, Italy
- Tommaso Schioppo
- Università degli studi di Milano, Milan, Italy
- Carolina Artusi
- Università degli studi di Milano, Milan, Italy
- Carlo Chizzolini
- Hospitaux Universitaires de Genève, Switzerland
- Aleksandra Zuber
- Hospitaux Universitaires de Genève, Switzerland
- Donatienne Wynar
- Hospitaux Universitaires de Genève, Switzerland
- Attila Balog
- University of Szeged, Szeged, Hungary
- Magdolna Deák
- University of Szeged, Szeged, Hungary
- Márta Bocskai
- University of Szeged, Szeged, Hungary
- Sonja Dulic
- University of Szeged, Szeged, Hungary
- Gabriella Kádár
- University of Szeged, Szeged, Hungary
- Falk Hiepe
- Charite, Berlin, Germany
- Silvia Thiel
- Charite, Berlin, Germany
- Manuel Rodriguez Maresca
- Andalusian Public Health System Biobank, Granada, Spain
- Antonio López-Berrio
- Andalusian Public Health System Biobank, Granada, Spain
- Rocío Aguilar-Quesada
- Andalusian Public Health System Biobank, Granada, Spain
- Héctor Navarro-Linares
- Andalusian Public Health System Biobank, Granada, Spain
- DOI
- https://doi.org/10.1016/j.jtauto.2025.100296
- Journal volume & issue
-
Vol. 11
p. 100296
Abstract
Objectives: To gain insights into the pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) and identify potential drug targets through investigation of whole-blood human transcriptome. Methods: We analysed differentially expressed genes in peripheral blood from active central nervous system (CNS) lupus (n = 26) and active non-neuropsychiatric SLE (n = 38) patients versus healthy controls (n = 497) from the European PRECISESADS project (NTC02890121). We further explored dysregulated gene modules in active CNS lupus and their correlation with serological markers. Lastly, we performed regulatory network and druggability analysis. Results: Unsupervised weighted gene co-expression network analysis (WGCNA) revealed 23 dysregulated gene modules and two subgroups of active CNS lupus. The interferon gene module was prominently upregulated in subgroup 1, while the B cell, T cell, and cytotoxic/natural killer (NK) cell modules were downregulated. Subgroup 2 showed less marked dysregulation patterns. Subgroup 1 had lower estimated proportions of lymphoid cell subsets and proportionally more patients positive for anti-dsDNA antibodies compared to subgroup 2, pointing to molecularly distinct subgroups or misclassification of subgroup 2. In silico prediction algorithms demonstrated a greater anticipated response to anifrolumab, C3 inhibitors, and calcineurin inhibitors for patients in CNS lupus subgroup 1 compared with subgroup 2. Conclusions: Gene dysregulation patterns related to innate and adaptive lymphoid immunity separated active CNS lupus patients into two distinct subgroups with differential anticipated response to type I interferon, C3, and calcineurin inhibition. Our study provides a conceptual framework for precision medicine in NPSLE and implications for overcoming the major clinical challenge of attributing neuropsychiatric features to SLE versus other causes.
Keywords
- Systemic lupus erythematosus
- Neuropsychiatric systemic lupus erythematosus
- Precision medicine
- Druggability
- Biologics
- Transcriptome