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Primary immune deficiency screening in patients with biopsy proven autoimmune liver diseases

Volume 30 · Issue Supplement 1 · July 2019 Publish Date: July 25, 2019
Cem Simsek 1 Javanşir Vahabov 2 Fuad Mustafayev 3 Alper Baysal 4 Deniz Nazire Çağdaş Ayvaz 5 Tuğrul Pürnak 6 Halis Şimşek 7 Yasemin Balaban 8
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Turkish Journal of Gastroenterology

Volume 30 · Issue Supplement 1 · July 2019

Pages: 91-91

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Published Online: July 25, 2019

Copyright © 2019 Cem Simsek, Javanşir Vahabov, Fuad Mustafayev, Alper Baysal, Deniz Nazire Çağdaş Ayvaz, Tuğrul Pürnak, Halis Şimşek, Yasemin Balaban

Cem Simsek
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Javanşir Vahabov
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Fuad Mustafayev
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Alper Baysal
Hacettepe University Faculty of Medicine Ankara Turkey
Deniz Nazire Çağdaş Ayvaz
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Tuğrul Pürnak
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Halis Şimşek
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Yasemin Balaban
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Simsek, C., Vahabov, J., Mustafayev, F., Baysal, A., Çağdaş Ayvaz, D. N., Pürnak, T., … Balaban, Y. (2019). Primary immune deficiency screening in patients with biopsy proven autoimmune liver diseases. Turkish Journal of Gastroenterology, 30(Supplement 1), 91–91. https://doi.org/10.5152/tjg.2019.60
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Abstract

INTRODUCTION: Autoimmune liver diseases (ALD) includes autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) which are chronic auto-inflammatory diseases of the liver with complex and heterogeneous pathogenesis. Malfunctioning immune system is the sine que non of ALD, and affects liver and other organs. We aimed to delineate the frequency of primary immune deficiency (PID) in patients with biopsy proven ALD.  METHODS: Single-institutional liver biopsy database, along with chart data was retrospectively evaluated between January 2007 and January 2018. The cases with a designated diagnosis of AIH, PBC, PSC and overlap syndrome (OS) were included. Data collection included age, sex, liver tests, autoantibody profiles, leukocyte and thrombocyte counts, immunoglobulin levels and biopsy parameters (hepatic activity index (HAI) and METAVIR fibrosis score (FS)). Leukopenia, neutropenia, lymphopenia and thrombocytopenia were defined as smaller than 4,4x103 cells/microL, 1,5x103 cells/microL, 1,0x103 cells/microL, and 1,5x106 cells respectively. PID was screened by presence of lymphopenia (RESULTS: ALD was confirmed in 60 cases out of 104 liver biopsy. The distribution of ALD was 37 (61.6%) AIH, 18 (30.0%) PBC, 3 (5%) PSC, and 2 (3.3%) OS. The median age was 47 years and 80.3% was woman. FS F1, F2, F3 and F4 were reported in 7 (11.6%), 18 (30.0%), 19 (31.6%), and 16 (26.8%) patients. Basal thrombocytopenia was reported in 15% of patients, and was not related to FS as 4/10 thrombocytopenic patients had F0 and F1 FS. The lymphopenia was detected 5 (8.3%) of ALD (8.1% in AIH, 5.6% in PBS, 50% in PSC, and 33.3% in OS). Leukopenia, neutropenia, and lymphopenia were respectively present in 13%, 8%, and 22% of patients. The lymphopenia was not associated with type of ALD, leukopenia, thrombocytopenia, HAI or FS. IgA levels were >70 mg/dL in all cases, however 6% were below 90 mg/dL. IgG and IgM levels were above lower limit in all cases. Leukopenia was seen in 13%, neutropenia in 8% and lymphopenia in 22%.  CONCLUSION: Although most of patients (88.4%) advanced fibrosis, leukopenia (13%) or thrombocytopenia (15%) was not frequent in this ALD cohort. Basal lymphopenia was present in approximately one-fifth of patients before immunosuppressive treatment. Lymphopenia was not associated with neither clinical or histological parameters. The immune dysregulation accompanying ALD warrants further investigation because of their well-known clinical and prognostic implications

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Article Info
Published In
Journal Turkish Journal of Gastroenterology
Volume / Issue Volume 30 · Issue Supplement 1 · July 2019
Pages 91-91
DOI 10.5152/tjg.2019.60
History
Published Online July 25, 2019
Copyright
Copyright (c) 2019 Cem Simsek, Javanşir Vahabov, Fuad Mustafayev, Alper Baysal, Deniz Nazire Çağdaş Ayvaz, Tuğrul Pürnak, Halis Şimşek, Yasemin Balaban
Affiliations
1
Cem Simsek
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
2
Javanşir Vahabov
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
3
Fuad Mustafayev
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
4
Alper Baysal
Hacettepe University Faculty of Medicine Ankara Turkey
5
Deniz Nazire Çağdaş Ayvaz
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
6
Tuğrul Pürnak
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
7
Halis Şimşek
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
8
Yasemin Balaban
Gastroenterology and Hepatology Hacettepe University Faculty of Medicine Ankara Turkey
Cite this Article
Simsek, C., Vahabov, J., Mustafayev, F., Baysal, A., Çağdaş Ayvaz, D. N., Pürnak, T., … Balaban, Y. (2019). Primary immune deficiency screening in patients with biopsy proven autoimmune liver diseases. Turkish Journal of Gastroenterology, 30(Supplement 1), 91–91. https://doi.org/10.5152/tjg.2019.60
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