Publikasi Scopus FKUI 2021 per tanggal 31 Juli 2021 (507 artikel)

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. © 2021 Pakistan Medical Association. All rights reserved.