Bite of the wolf: innate immune responses propagate autoimmunity in lupus

The etiopathogenesis of systemic lupus erythematosus (SLE), a clinically heterogeneous multisystemic syndrome that derives its name from the initial characterization of facial lesions that resemble the bite of a wolf, is considered a complex, multifactorial interplay between underlying genetic susce...

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Bibliographic Details
Published in:The Journal of clinical investigation 2021-02, Vol.131 (3)
Main Authors: Gupta, Sarthak, Kaplan, Mariana J
Format: Article
Language:English
Subjects:
Adaptive Immunity
Animals
Autoimmunity
Cell death
Development and progression
Health aspects
Humans
Immune response
Immunity, Innate
Interferons - immunology
Lupus Erythematosus, Systemic - immunology
Lupus Erythematosus, Systemic - pathology
Natural immunity
Neutrophils - immunology
Neutrophils - pathology
Review
Systemic lupus erythematosus
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Summary:The etiopathogenesis of systemic lupus erythematosus (SLE), a clinically heterogeneous multisystemic syndrome that derives its name from the initial characterization of facial lesions that resemble the bite of a wolf, is considered a complex, multifactorial interplay between underlying genetic susceptibility factors and the environment. Prominent pathogenic factors include the induction of aberrant cell death pathways coupled with defective cell death clearance mechanisms that promote excessive externalization of modified cellular and nuclear debris with subsequent loss of tolerance to a wide variety of autoantigens and innate and adaptive immune dysregulation. While abnormalities in adaptive immunity are well recognized and are key to the pathogenesis of SLE, recent findings have emphasized fundamental roles of the innate immune system in the initiation and propagation of autoimmunity and the development of organ damage in this disease. This Review focuses on recent discoveries regarding the role of components of the innate immune system, specifically neutrophils and interferons, in promoting various aspects of lupus pathogenesis, with potential implications for novel therapeutic strategies.
ISSN:0021-9738
1558-8238
DOI:10.1172/JCI144918