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Erschienen in: Wiener klinische Wochenschrift 5-6/2022

20.07.2021 | original article

Seronegative autoimmune hepatitis in children

A real diagnostic challenge

verfasst von: MD Mohammed A. Khedr, MD Tahany A. Salem, MD Ghada M. Boghdadi, MD Ahmed S. Elharoun, MD Allia A. El-Shahaway, MD Hany R. Atallah, MD Mostafa M. Sira

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 5-6/2022

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Summary

Background and aim

Classical autoimmune hepatitis (AIH) is characterized by the presence of conventional autoantibodies (anti-smooth muscle, antinuclear and anti-liver-kidney-microsomal antibodies). The absence of such autoantibodies in some patients does not preclude AIH diagnosis or the need for its treatment. This group of patients was termed seronegative AIH. Whether non-conventional autoantibodies can identify this group of patients is still elusive. We aimed to study the prevalence of seronegativity of conventional autoantibodies and the occurrence of non-conventional autoantibodies in children with AIH.

Methods

In this study, 55 children with AIH were investigated for non-conventional autoantibodies (anti-neutrophil cytoplasmic antibodies, antibodies to soluble liver antigen, anti-tissue transglutaminase and antiplatelet antibodies). All the patients received immunosuppressive therapy and were assessed for treatment response.

Results

Of the patients 44 had classical AIH (type 1, 70.09%, type 2, 9.09%) and 20% were seronegative. The four studied non-conventional autoantibodies occurred in four patients, one for each. All non-conventional autoantibodies were exclusively associated with type 1 AIH. The clinical profile, ultrasonographic findings, liver biochemistry and histopathological findings were comparable in the classical and seronegative AIH. The majority of patients with classical (72.7%) and seronegative (54.5%) AIH were treatment responders.

Conclusion

Seronegative AIH represents a substantial percentage of pediatric patients diagnosed with AIH. They were even negative for non-conventional autoantibodies. Furthermore, apart from autoantibodies, seronegative AIH is almost indistinguishable from the classical AIH and the majority of patients were treatment responders. This favorable response to immunosuppression deserves sustainable efforts for considering such a diagnosis and start therapy to halt disease progression is worthwhile.
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Metadaten
Titel
Seronegative autoimmune hepatitis in children
A real diagnostic challenge
verfasst von
MD Mohammed A. Khedr
MD Tahany A. Salem
MD Ghada M. Boghdadi
MD Ahmed S. Elharoun
MD Allia A. El-Shahaway
MD Hany R. Atallah
MD Mostafa M. Sira
Publikationsdatum
20.07.2021
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 5-6/2022
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-021-01907-x