Z Gastroenterol 2018; 56(01): E2-E89
DOI: 10.1055/s-0037-1612697
Poster Visit Session II Clinical Hepatology – Friday, January 26, 2018, 2:35pm – 3:20pm, Room 120
Georg Thieme Verlag KG Stuttgart · New York

Autoimmune hepatitis with normal IgG at diagnosis: Subtype without selective elevation of IgG but similar histological features and treatment response

J Hartl
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
G Wong
2   King's College, Institute of Liver Studies, London
,
K Zachou
3   Universtiy of Larissa, Department of Medicine and Research Laboratory of Internal Medicine, Larissa
,
A Ashgar
4   University of Birmingham, Institute of Liver Studies, Birmingham
,
S Pape
5   Radboud University Medical Center, Department of Gastroenterology and Hepatology, Nijmegen
,
M Sebode
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
M Peiseler
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
C Weiler-Normann
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
R Zenouzi
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
T Krech
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
H Ehlken
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
S Huebscher
4   University of Birmingham, Institute of Liver Studies, Birmingham
,
R Miquel
2   King's College, Institute of Liver Studies, London
,
C Schramm
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
,
J Drenth
5   Radboud University Medical Center, Department of Gastroenterology and Hepatology, Nijmegen
,
Y Oo
4   University of Birmingham, Institute of Liver Studies, Birmingham
,
G Dalekos
3   Universtiy of Larissa, Department of Medicine and Research Laboratory of Internal Medicine, Larissa
,
M Heneghan
2   King's College, Institute of Liver Studies, London
,
A Lohse
1   University Hospital Hamburg-Eppendorf (UKE), I. Medical Centre, Hamburg
› Author Affiliations
Further Information

Publication History

Publication Date:
03 January 2018 (online)

Also available at
 

Background:

The presence of selectively elevated IgG levels is a hallmark of AIH and has found its way into diagnostic scores. Nevertheless, about 15% of patients show normal IgG levels. The clinical significance of normal IgG values at diagnosis has so far not been explored in detail.

Methods:

We assessed biochemical, clinical and histological features from AIH patients with normal IgG-values at diagnosis. Data from 5 European high-volume centres were included. Liver histology was re-assessed by a reference pathologist to assure the diagnosis and compare histology in more detail.

Results:

In total, 130 patients with normal IgG-levels at diagnosis and an age- and sex-matched control group (n = 130) were included (Hamburg: n = 31, London: n = 51, Larissa: n = 30; Nijmegen: n = 9, Birmingham: n = 9).

No difference of fibrosis stage (2.3 ± 1.2 vs. 2.3 ± 1.3) or grading (3.0 ± 0.89 vs. 3.0 ± 0.93) according to Desmet and Scheuer classification was observed.

Besides higher IgG levels (IgG at diagnosis: 12.2 ± 3.2 g/L vs. 29.5 ± 5.8 g/L; p < 0.0001), patients with typical AIH had also a higher IgG/IgA ratio (9.3 ± 6.9 g/L vs. 5.4 ± 2.4 g/L; p<.00001) than patients with normal IgG at diagnosis. Moreover, the IgG/IgA ratio significantly decreased after initiation of immunosuppression (p<.0001), while it remained stable in patients with normal IgG at diagnosis. Of note, the IgG/IgA ratio was no longer different between study groups after 12 months of treatment (“typical AIH”: 5.8 ± 4.2 vs. “normal IgG”: 5.5 ± 2.2; p = 0.36).

Besides IgG and IgA, there was no difference in biochemical markers between groups at diagnosis, after 12 months under immunosuppression or at last follow-up.

Interestingly, 27% of patients with normal IgG values at diagnosis could be successfully weaned from immunosuppression, compared to only 13% in the control group (p = 0.027) and remained in remission for at least 2 years.

Conclusions:

Patients with normal IgG at diagnosis present with similar biochemical and histological features and show comparable treatment response, but show no selective elevation of IgG.