{
  "abstract": "Introduction Cognitive symptoms such as ‘brain fog’ are frequent and disabling in patients with SLE. We aimed to develop and validate the Lupus Brain Fog Severity Scale (LBFSS), the first patient-reported outcome measure (PROM) for cognitive symptoms in SLE.Methods The LBFSS was developed using a multi-step international approach involving healthcare professionals and patients with SLE. Patients provided free-text descriptions of brain fog, generating candidate domains through thematic analysis (step 1). Domains were rated for relevance (step 2) and those retained converted into self-assessable items (step 3). The pilot LBFSS was iteratively refined (step 4). The finalised LBFSS was validated (step 5) in a large international SLE cohort. Psychometric evaluation included internal consistency, exploratory factor analysis and assessment of construct, convergent, known-groups and criterion-related validity.Results In step 1, 147 SLE participants across 39 countries generated 21 domains for cognitive symptoms, of which 13 were retained in consensus (step 2), converted into self-assessable items (step 3) and iteratively refined using patient and physician feedback. In step 5, validation of the LBFSS instrument in 378 patients from 36 countries demonstrated high internal consistency (Cronbach’s α=0.95) and a unidimensional structure explaining 62.2% of variance. LBFSS scores were significantly higher in participants reporting brain fog (p<0.0001), confirming known-group validity. Construct validity was supported by significant correlations with functional impact and symptom severity (ρ=0.46–0.65, p<0.0001), strong convergent validity with the Perceived Deficits Questionnaire-20 (ρ=0.84, p<0.0001) while the discriminative performance for presence versus absence of self-reported brain fog was high (area under the curve=0.85).Conclusion The LBFSS is a new, valid and feasible PROM to assess brain fog and cognitive symptoms in SLE.",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology, National Reference Center for Autoimmune Diseases (RESO), Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Strasbourg, France"
      ],
      "name": "Laurent Arnaud"
    },
    {
      "affiliations": [
        "Rheumatology Unity, AOU Cagliari, Department of Medical Science and Public Health, University of Cagliari, Cagliari, Italy"
      ],
      "name": "Matteo Piga"
    },
    {
      "affiliations": [
        "Regional Center for Autoimmune and Rheumatic Diseases (GO-CREAR), Rosario, Argentina"
      ],
      "name": "Guillermo J Pons-Estel"
    },
    {
      "affiliations": [
        "Departamento de Medicina, Universidad de Oriente, Ciudad Bolivar, Venezuela"
      ],
      "name": "Yurilis J Fuentes-Silva"
    },
    {
      "affiliations": [
        "Internal Medicine and Systemic Diseases Unit, University Hospital Centre Dijon, Dijon, France"
      ],
      "name": "Hervé Devilliers"
    },
    {
      "affiliations": [
        "Unidade de Imunologia Clínica – Unidade Local de Saúde Santo António",
        "UMIB - Unit for Multidisciplinary Research in Biomedicine, ICBAS - School of Medicine and Biomedical Sciences, University of Porto, Porto, Portugal",
        "ITR - Laboratory for Integrative and Translational Research in Population Health, Porto, Portugal"
      ],
      "name": "Raquel Faria"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Mayo Clinic Minnesota, Rochester, Minnesota, USA"
      ],
      "name": "Ali Duarte-Garcia"
    },
    {
      "affiliations": [
        "UDAI – Autoimmune Diseases and Immunology Unit, Internal Medicine Department, ULS Tâmega e Sousa, Penafiel, Portugal",
        "Unit for Multidisciplinary Research in Biomedicine (UMIB), School of Medicine and Biomedical Sciences (ICBAS), University of Porto, Porto, Portugal",
        "i3S – Instituto de Investigação e Inovação em Saúde, Universidade do Porto, Porto, Portugal"
      ],
      "name": "Daniel Guimarães de Oliveira"
    },
    {
      "affiliations": [
        "Rheumatology Section, Hospital italiano de Buenos Aires, Buenos Aires, Argentina"
      ],
      "name": "Marina Scolnik"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Radboud University Medical Center, Nijmegen, the Netherlands"
      ],
      "name": "Sander van Leuven"
    },
    {
      "affiliations": [
        "School of Infection and Immunity, University of Glasgow, Glasgow, UK"
      ],
      "name": "Eve Smith"
    },
    {
      "affiliations": [
        "Departmento of Orthopedics, Rheumatology and Traumatology-Universidade Estadual de Campinas, Campinas, Brazil"
      ],
      "name": "Simone Appenzeller"
    },
    {
      "affiliations": [
        "School of Medicine, University of Zagreb, Zagreb, Croatia",
        "Division of Clinical Immunology and Rheumatology, Referral Centre for SLE and Related Diseases, Department of Internal Medicine, University Hospital Centre Zagreb, Zagreb, Croatia"
      ],
      "name": "Ivan Padjen"
    },
    {
      "affiliations": [
        "Johns Hopkins University School of Medicine, Baltimore, Maryland, USA"
      ],
      "name": "Michelle A Petri"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliero-Universitaria S.Anna, Ferrara, Italy"
      ],
      "name": "Alessandra Bortoluzzi"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Center for Molecular Medicine (CMM), Stockholm, Sweden",
        "Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden"
      ],
      "name": "Ioannis Parodis"
    },
    {
      "affiliations": [
        "Feinstein Institutes for Medical Research, Northwell Health System, Manhasset, New York, USA"
      ],
      "name": "Meggan Mackay"
    },
    {
      "affiliations": [
        "Rheumatology Division, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia, Rumah Sakit Universitas Indonesia, Depok, Indonesia"
      ],
      "name": "Faisal Parlindungan"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Department of Internal Medicine, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil"
      ],
      "name": "Odirlei A Monticielo"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology Unit, Attikon University Hospital, National Kapodistrian University of Athens Medical School, Athens, Greece"
      ],
      "name": "Antonis Fanouriakis"
    },
    {
      "affiliations": [
        "Department of Neuropsychology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France"
      ],
      "name": "Catherine Kleitz"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Hospital for Sick Children, Toronto, Ontario, Canada",
        "Neurosciences and Mental Health Program, SickKids Research Institute, Toronto, Ontario, Canada",
        "Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada"
      ],
      "name": "Andrea M Knight"
    },
    {
      "affiliations": [
        "Department of Neuropsychology, Hôpitaux Universitaires de Strasbourg, Strasbourg, France"
      ],
      "name": "Nathalie Philippi"
    },
    {
      "affiliations": [
        "School of Clinical Sciences, Monash University, Melbourne, Victoria, Australia"
      ],
      "name": "Alberta Hoi"
    },
    {
      "affiliations": [
        "Lupus Europe, Brussels, Belgium"
      ],
      "name": "Zoe Karakikla-Mitsakou"
    },
    {
      "affiliations": [
        "Center for Research and Social Intervention (CIS-Iscte), ISCTE-University Institute of Lisbon, Lisbon, Portugal"
      ],
      "name": "Sofia Silva Ribeiro"
    },
    {
      "affiliations": [
        "Lupus Europe, Brussels, Belgium"
      ],
      "name": "Annemarie Sluijmers"
    },
    {
      "affiliations": [
        "Lupus Europe, Brussels, Belgium"
      ],
      "name": "Aldevina Sturiene"
    },
    {
      "affiliations": [
        "Lupus Program at University Health Network, Schroeder Arthritis Institute, Krembil Research Institute, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada"
      ],
      "name": "Zahi Touma"
    }
  ],
  "title": "International development of a lupus-specific instrument to assess cognitive symptoms in patients with SLE: Lupus Brain Fog Severity Scale (LBFSS) study",
  "uid": "91080318-9c17-51b1-b0a5-831521c1bd0a"
}