Coexisting systemic lupus erythematosus and suspected hyperimmunoglobulin E syndrome: A case report

Hyperimmunoglobulin E syndrome (HIES) is a rare multisystem syndrome that affects musculoskeletal system, connective tissue, and immune system. Immune dysregulation in hyperimmunoglobulin E syndrome often accompanied by autoimmune diseases, such as systemic lupus erythematosus. While the role of IgG...

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Bibliographic Details
Published in:Journal of the Pakistan Medical Association 2021-02, Vol.71(Suppl 2) (2), p.S140-S142
Main Author: Tjahjadi, Ricky
Format: Article
Language:English
Subjects:
Adult
Autoantibodies
Autoimmune Diseases
Case studies
Comorbidity
Female
Humans
Immunological deficiency syndromes
Lupus Erythematosus, Systemic - complications
Lupus Erythematosus, Systemic - diagnosis
Systemic lupus erythematosus
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Summary:Hyperimmunoglobulin E syndrome (HIES) is a rare multisystem syndrome that affects musculoskeletal system, connective tissue, and immune system. Immune dysregulation in hyperimmunoglobulin E syndrome often accompanied by autoimmune diseases, such as systemic lupus erythematosus. While the role of IgG autoantibody is well known in the pathogenesis of systemic lupus erythematosus, other subtypes of autoantibodies, IgE in particular, are now known to be involved in autoimmune process. A female with known elevated IgE was diagnosed with adult onset HIES. Atypical chest pain and otherwise normal cardiac imaging studies prompted her for further lab testings. Positive antinuclear antibody immunofluorescence test and elevated anti ds-DNA led to SLE diagnosis. Lack of characteristic clinical manifestations complicates establishing HIES while showing the extent to which IgE elevation could be related to SLE.
ISSN:0030-9982