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. 2023 Nov;34(Suppl2):S1-S33.
doi: 10.5152/tjg.2023.23242.

Diagnosis and Treatment of Autoimmune Hepatitis: Questions, Answers, and Illustrative Cases: Endorsed by Autoimmune Liver Diseases Special Interest Group, Turkish Association for the Study of Liver

Ersan Özaslan  1 , Fulya Günşar  2 , Aslı Çiftçibaşı Örmeci  3 , İbrahim Hatemi  4 , Cumali Efe  5 , Gülen Akyol  6 , Nesrin Turhan  7 , Funda Yılmaz Barbet  8 , Özgül Sağol  9 , Çiğdem Ataizi Çelikel  10 , Mine Güllüoğlu  11 , Dilara Turan Gökçe  12 , Zülal İstemihan  3 , Tuğçe Eşkazan  4 , Ramazan İdilman  13
Affiliations

Diagnosis and Treatment of Autoimmune Hepatitis: Questions, Answers, and Illustrative Cases: Endorsed by Autoimmune Liver Diseases Special Interest Group, Turkish Association for the Study of Liver

Ersan Özaslan et al. Turk J Gastroenterol. 2023 Nov.

Abstract

Autoimmune hepatitis (AIH) is a rare, immune-mediated liver disease. It has a heterogeneous nature with varied clinical presentations. The management of patients with AIH is challenging in many ways. The main difficulties are inexperience due to the rarity of the disease, diagnostic confusion in controversial areas such as variant/overlap cases, acute presentations, the presence of non-alcoholic fatty liver disease or drug-induced liver injury features, and the long and complex course of treatment. Here, we provide a clear, concise, and visualized review regarding the diagnosis and treatment of AIH, including illustrative cases.

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Conflict of interest statement

Declaration of Interests: Authors declare no conflict of interests for this article.

Figures

Figure 1.
Figure 1.
The clinical spectrum of autoimmune hepatitis.
Figure 2.
Figure 2.
The diagnostic approach to autoimmune hepatitis .
Figure 3.
Figure 3.
The illustration of characteristic histological findings in acute and chronic hepatitides (red circles, apoptotic hepatocytes; blue zones, fibrosis).
Figure 4.
Figure 4.
Treatment is indicated in active disease (green box), in selected cases of mild disease (yellow box), and it is not recommended in extremes (red boxes).
Figure 5.
Figure 5.
The definition of treatment responses in autoimmune hepatitis (adopted from the consensus report, initial response and relapse were added for didactic purposes, positive responses are shown in the upper set and negative responses are shown in the lower set).
Figure 6.
Figure 6.
The phases of a typical treatment course in a classical responder patient with AIH. The first-line treatment options, serum ALT, IgG and histological inflammation is illustrated on the longitudinal time scale. AIH, autoimmune hepatitis; ALT, alanine aminotransferase; CBR, complete biochemical response; IgG, immunoglobulins.
Figure 7.
Figure 7.
The first-line treatment of AIH. Source: Adopted from AASLD 2019 guideline. *Classical AIH includes chronic or acute cases with mild-to-moderate severity and cirrhotic cases. AIH, autoimmune hepatitis; IST, immunosuppressive therapy; TPMT, thiopurine methyl transferase.
Figure 8.
Figure 8.
Some illustrative examples of insufficient response spectrum are shown on the longitudinal time scale of autoimmune hepatitis.
Figure 9.
Figure 9.
The second- and third-line treatment options in autoimmune hepatitis.
Figure 10.
Figure 10.
Heterogenous nature of autoimmune liver diseases.
Figure 11.
Figure 11.
The diagnostic criteria of classical autoimmune liver diseases (upper box) and the origins of variant/overlap phenotypes (lower box). (*Liver biopsy for PBC: Serology negative or marked hepatitic features. *Liver biopsy for PSC: Small-duct involvement or marked hepatitic features.)
Figure 12.
Figure 12.
The illustration of typical (upper box) and compatible (lower boxes) histological features of classical autoimmune liver diseases.
Figure 13.
Figure 13.
The immune-mediated drug-induced liver injury phenotypes that can resemble idiopathic autoimmune hepatitis.
See this image and copyright information in PMC

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