The mosaic of systemic lupus erythematosus: From autoimmunity to autoinflammation and immunodeficiency and back.

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  • Additional Information
    • Source:
      Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128967 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-0183 (Electronic) Linking ISSN: 15689972 NLM ISO Abbreviation: Autoimmun Rev Subsets: MEDLINE
    • Publication Information:
      Original Publication: Amsterdam ; New York : Elsevier, c2002-
    • Subject Terms:
      Lupus Erythematosus, Systemic*/immunology ; Lupus Erythematosus, Systemic*/genetics ; Lupus Erythematosus, Systemic*/diagnosis ; Autoimmunity*/immunology; Humans ; Inflammation/immunology ; Animals ; Immunologic Deficiency Syndromes/immunology ; Immunologic Deficiency Syndromes/diagnosis ; Immunologic Deficiency Syndromes/genetics ; Immunity, Innate ; Inflammasomes/immunology ; Adaptive Immunity
    • Abstract:
      The concept of an "immunological continuum model," introduced by McGonagle and McDermott in 2006, redefines the traditional dichotomy between autoimmunity and autoinflammation, proposing a spectrum where innate and adaptive immune dysregulation can co-occur, reflecting a more nuanced understanding of immune disorders. Systemic lupus erythematosus (SLE) exemplifies the complexity of this continuum, often displaying manifestations of autoimmunity, autoinflammation, and immunodeficiency. The interplay between genetic, epigenetic, hormonal, psychological, and environmental factors contributes to its distinctive immunopathological signatures. Historically recognized as a systemic disease with diverse clinical manifestations, SLE is primarily a polygenic autoimmune condition but can, however, present in monogenic forms. Examining SLE through the lens of the immunological continuum model allows for emphasis on the contributions of both innate and adaptive immunity. SLE and primary immunodeficiencies share genetic susceptibilities and clinical manifestations. Additionally, autoinflammatory mechanisms, such as inflammasome activation and interferonopathies, can play a role in SLE pathogenesis, illustrating the disease's position at the crossroads of immune dysregulation. Recognizing the diverse clinical expressions of SLE and its mimickers is critical for accurate diagnosis and targeted therapy. In conclusion, the immunological continuum model provides a comprehensive framework for understanding SLE, acknowledging its multifaceted nature and guiding future research and clinical practice toward more effective and individualized treatments. After the Mosaic of Autoimmunity, it is now the time to focus and attempt to solve the intricate mosaic of SLE.
      Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
      (Copyright © 2024 Elsevier B.V. All rights reserved.)
    • Contributed Indexing:
      Keywords: Autoimmunity; Autoinflammation; Immunodeficiency; Monogenic lupus; SLE; Systemic lupus erythematosus
    • Accession Number:
      0 (Inflammasomes)
    • Publication Date:
      Date Created: 20241031 Date Completed: 20241128 Latest Revision: 20241205
    • Publication Date:
      20241209
    • Accession Number:
      10.1016/j.autrev.2024.103675
    • Accession Number:
      39481623