Роль антинейтрофильных цитоплазматических антител класса Ig G в диагностике аутоиммунногогепатита 1-го типа у детей: ОРИГИНАЛЬНЫЕ СТАТЬИ

Анастасия Руслановна  Королева; Дарья Александровна  Кузнецова; Сергей Владимирович  Лапин; Лариса Георгиевна  Горячева; Вера Александровна  Грешнякова; Екатерина Анатольевна  Уланова; Маргарита Юрьевна  Первакова

doi:10.56871/PED.2025.36.25.003

The role of antineutrophil cytoplasmic antibodiesof the Ig G class in the diagnosis of autoimmune hepatitistype 1 in children

ORIGINAL STUDIES

Anastasia R. Koroleva Pavlov First Saint Petersburg State Medical University https://orcid.org/0009-0002-1413-5224
Daria A. Kuznetsova Pavlov First Saint Petersburg State Medical University https://orcid.org/0000-0001-5318-354X
Sergey V. Lapin Pavlov First Saint Petersburg State Medical University https://orcid.org/0000-0002-4998-3699
Larisa G. Goryacheva Saint Petersburg State Pediatric Medical University; Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency https://orcid.org/0000-0001-7890-733X
Vera A. Greshnyakova Saint Petersburg State Pediatric Medical University; Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency; Saint Petersburg State University https://orcid.org/0000-0002-4509-5352
Ekaterina A. Ulanova Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency https://orcid.org/0009-0003-1143-7339
Margarita Y. Pervakova Pavlov First Saint Petersburg State Medical University https://orcid.org/0000-0001-9630-257X

Keywords:

antineutrophil cytoplasmic antibodies autoimmune hepatitis type 1 in children antigen specificity neutrophil elastase smooth muscle antibodies biochemical activity of autoimmune hepatitis type 1 elastography антинейтрофильные цитоплазматические антитела аутоиммунный гепатит 1-го типа у детей антигенная специфичность нейтрофильная эластаза антитела к гладким мышцам биохимическая активность аутоиммунного гепатита 1-го типа эластография

Abstract

Background.The serological profile of autoimmune hepatitis (AIH) includes a wide range of markers, including antineutrophil cytoplasmic antibodies (ANCA), but the clinical and diagnostic role of ANCA has not been definitively established.

Aim.To determine the incidence and antigen specificity of ANCA Ig G in children with AIH type 1 (AIH-1) and the correlation of ANCA Ig G with serological and biochemical parameters of AIH-1 in children.

Materials and methods.The study included 38 patients withAIH-1and 5 patients withAIH type 2 (AIH-2). Autoantibodies were detected by indirect immunofluorescence, the antigen specificity of ANCA Ig G was determined by enzyme linked immunosorbent assay, the level of total Ig G was determined by the turbidimetric method.

Results.The prevalence of ANCA Ig G in patients with AIH-1 was 81.6%, smooth muscle antibodies (SMA) — 84.2%. Combined detection of ANCA Ig G and SMA was observed in 40,8% of cases, only ANCA Ig G was detected in 25.9% of cases. At the onset of AIH-1 SMA and ANCA Ig G were detected in 64% of patients, while in 8% of cases only ANCA Ig G was detected. When determining the antigen specificity of ANCA Ig G, neutrophil elastase was the molecular target in 43.8% of patients. There was a moderate correlation of ANCA Ig G with SMA and the biochemical activity of AIH-1, a weak correlation was found with the results of elastography and total Ig G, and a noticeable correlation was found with gamma-glutamyl transpeptidase (GGTP). Differences in the results of SMA, alanine aminotransferase (ALT), aspartate aminotransferase (AST) and GGTP were found in the ANCA-seropositive and ANCA-seronegative groups. In 13 patients with a tendency to decrease the titers of ANCA Ig G and SMA, a significant decrease in ALT, AST, GGTP was found.

Conclusions.The incidence of ANCA Ig G is comparable to the incidence of SMA in AIH-1 in children, in half of the cases of the disease these autoantibodies are detected in combination. Neutrophil elastase is the most common antigenic target of ANCA Ig G in children with AIH-1. ANCA Ig G titers directly correlate with SMA, ALT, AST, GGTP. A positive ANCA Ig G test result may be the only serological marker for AIH-1 in children.

Author Biographies

Anastasia R. Koroleva, Pavlov First Saint Petersburg State Medical University

Clinical Laboratory
Diagnostics Specialist, Laboratory of Diagnostics
of Autoimmune Diseases, Center of Molecular Medicine

Daria A. Kuznetsova, Pavlov First Saint Petersburg State Medical University

MD, Ph D, Leading Scientist,
Laboratory of Diagnostics of Autoimmune Diseases,
Center of Molecular Medicine

Sergey V. Lapin, Pavlov First Saint Petersburg State Medical University

MD, Ph D, Head, Laboratory
of Diagnostics of Autoimmune Diseases, Center of Molecular Medicine

Larisa G. Goryacheva, Saint Petersburg State Pediatric Medical University; Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency

MD, Ph D, Dr Med Sci,
Professor, Department of Infectious Diseases in Children; Leading Scientist, Department of Viral Hepatitis
and Liver Diseases

Vera A. Greshnyakova, Saint Petersburg State Pediatric Medical University; Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency; Saint Petersburg State University

Associate
Professor, Department of Infectious Diseases in Children; MD, Ph D, Head, Department
of Viral Hepatitis and Liver Diseases; Associate Professor, Department of Infectious Diseases

Ekaterina A. Ulanova, Federal Scientific and Clinical Center of Infectious Diseases of the Federal Medical-Biological Agency

Junior Researcher, Department
of Viral Hepatitis and Liver Diseases

Margarita Y. Pervakova, Pavlov First Saint Petersburg State Medical University

MD, Ph D, Clinical Laboratory Diagnostics Specialist, Laboratory of Diagnostics of Autoimmune Diseases, Center of Molecular Medicine

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