@article{3104595,
    title = "Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the ‘Attikon’ cohort",
    author = "Nikolopoulos, D. and Kostopoulou, M. and Pieta, A. and Karageorgas, T. and Tseronis, D. and Chavatza, K. and Flouda, S. and Rapsomaniki, P. and Banos, A. and Kremasmenou, E. and Tzavara, V. and Katsimbri, P. and Fanouriakis, A. and Boumpas, D.T.",
    journal = "Lupus Science and Medicine",
    year = "2020",
    volume = "29",
    number = "5",
    pages = "514-522",
    publisher = "SAGE Publications Ltd",
    doi = "10.1177/0961203320908932",
    keywords = "azathioprine;  belimumab;  calcineurin inhibitor;  cyclophosphamide;  double stranded DNA antibody;  glucocorticoid;  hydroxychloroquine;  lupus anticoagulant;  methotrexate;  mycophenolate mofetil;  phospholipid antibody;  rituximab, adult;  alopecia;  arthritis;  Article;  autoimmune hemolytic anemia;  Caucasian;  clinical assessment;  cohort analysis;  disease burden;  disease duration;  disease severity;  drug hypersensitivity;  drug withdrawal;  eye toxicity;  female;  fever;  follow up;  human;  immunosuppressive treatment;  incidence;  inflammation;  leukopenia;  livedo reticularis;  lupus erythematosus nephritis;  lymphadenopathy;  major clinical study;  male;  mental disease;  mouth ulcer;  nose ulcer;  onset age;  phenotype;  photosensitivity;  priority journal;  Raynaud phenomenon;  skin lupus erythematosus;  systemic lupus erythematosus;  thrombocytopenia;  thrombosis;  brain vasculitis;  Caucasian;  classification;  comorbidity;  Greece;  incidence;  lupus erythematosus nephritis;  middle aged;  phenotype;  retrospective study;  rheumatology;  severity of illness index;  systemic lupus erythematosus;  young adult, Adult;  Comorbidity;  European Continental Ancestry Group;  Female;  Greece;  Humans;  Incidence;  Lupus Erythematosus, Systemic;  Lupus Nephritis;  Lupus Vasculitis, Central Nervous System;  Male;  Middle Aged;  Phenotype;  Retrospective Studies;  Rheumatology;  Severity of Illness Index;  Young Adult",
    abstract = "Objective: This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at ‘Attikon’ University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece. Methods: This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation. Results: The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common ‘classification’ manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud’s phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide. Conclusion: In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management. © The Author(s) 2020."
}