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First Diagnosis of Systemic Lupus Erythematosus in Hospitalized Patients: Clinical Phenotypes and Pitfalls for the Non-Specialist

  • Noemin Kapsala
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • Dionysis Nikolopoulos
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece

    Laboratory of Immune Regulation and Tolerance, Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece
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  • Sofia Flouda
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • Aikaterini Chavatza
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • Dimitrios Tseronis
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • Michail Aggelakos
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • Pelagia Katsimbri
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece
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  • George Bertsias
    Affiliations
    Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Heraklion, Crete, Greece

    Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Heraklion, Crete, Greece
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  • Antonis Fanouriakis
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece

    Department of Rheumatology, "Asklepieion" General Hospital, Voula, Athens, Greece
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  • Dimitrios T. Boumpas
    Correspondence
    Requests for reprints should be addressed to Dimitrios T. Boumpas, MD, Biomedical Research Foundation Academy of Athens, 4 Soranou Ephessiou St., 115 27 Athens, Greece.
    Affiliations
    "Attikon" University Hospital of Athens, Rheumatology and Clinical Immunology, Medical School, National and Kapodistrian University of Athens, Athens, Greece

    Laboratory of Immune Regulation and Tolerance, Autoimmunity and Inflammation, Biomedical Research Foundation of the Academy of Athens, Athens, Greece

    Medical School, University of Cyprus, Nicosia, Cyprus
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      Abstract

      Background

      Prompt recognition of systemic lupus erythematosus (SLE) in hospitalized patients presenting with severe disease is essential to initiate treatment. We sought to characterize the phenotype of hospitalized patients with new-onset SLE and estimate potential diagnostic delays.

      Methods

      An observational study of 855 patients (“Attikon” SLE cohort). Clinical phenotype was categorized according to the leading manifestation that led to hospitalization. Disease features, time to diagnosis, classification criteria, and the SLE Risk Probability Index (SLERPI) were recorded for each patient.

      Results

      There were 191 patients (22.3% of the total cohort) hospitalized due to manifestations eventually attributed to SLE. Main causes of admission were neuropsychiatric syndromes (21.4%), cytopenias (17.8%), nephritis (17.2%), and thrombotic events (16.2%). Although 79.5% of patients were diagnosed within 3 months from hospitalization, in 39 patients diagnosis was delayed, particularly in those with hematological manifestations. At hospitalization, a SLERPI >7 (indicating high probability for SLE) was found in 87.4% of patients. Patients missed by the SLERPI had fever, thrombotic or neuropsychiatric manifestations not included in the algorithm. Lowering the SLERPI threshold to 5 in patients with fever or thrombotic events increased the diagnostic rate from 88.8% to 97.9% in this subgroup, while inclusion of all neuropsychiatric events yielded no additional diagnostic value.

      Conclusion

      One in five patients with new-onset SLE manifest disease presentations required hospitalization. Although early diagnosis was achieved in the majority of cases, in approximately 20%, diagnosis was delayed. A lower SLERPI cut-off (≥5) in patients with fever or thrombosis could enhance early diagnosis.

      Keywords

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