Ersan Ozaslan

Otoimmün hepatit (OİH), nadir görülen ve immün sistem aracılı bir karaciğer hastalığı olup, klinik spekturumu oldukça geniştir. OİH tanısı, hastalığın seyrek görülmesi, klinik belirtilerinin diğer karaciğer hastalıklarıyla karışabilmesi ve spesifik laboratuvar testlerinin gerekmesi nedeniyle zorluklar içermektedir. Hastalığın patogenezi tam olarak anlaşılamamış olsa da, genetik yatkınlık ve çevresel faktörlerin rol oynadığı düşünülmektedir. Tedavide, hastalık aktivitesinin kontrol altına alınması ve remisyonun sağlanması hedeflenir. Steroidler, azatiyoprin ve diğer immünosupresif ilaçlar tedavinin temel taşlarını oluşturur. İlk aşama tedaviye yanıt vermeyen veya tedaviye intoleransı olan hastalar için alternatif tedavi stratejileri gerekmektedir. Bu bölümde, otoimmun hepatitin tanı ve tedavisindeki yaklaşımları, klinik uygulamaları ve tedaviye yanıt süreçleri yanı sıra akut şiddetli hepatit ve kronik zeminde akut karaciğer yetmezliği gibi özel durumlara yer verilecektir.

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.

The performance of non-invasive fibrosis markers has not been well studied in autoimmune hepatitis (AIH). Acoustic radiation force impulse imaging (ARFI) is a non-invasive radiological method for assessment of liver fibrosis. The aim of this study was to evaluate the accuracy of ARFI for fibrosis staging in patients with AIH. AIH patients who undergone for control liver biopsy after at least two years of biochemical remission period were also assessed by ARFI. Liver fibrosis was staged according to the METAVIR scoring system. A total of 15 patients (13 women and 2 men) with mean age of 40.9 (18-59 years) were included in the study. Repeat liver biopsy was performed a mean of 4.9 (3-9 years) after AIH diagnosis. Nine patients had significant fibrosis scores (F ≥ 2) while, remain 6 patients had mild or no fibrosis. The mean (±SD) shear wave velocities for patients with fibrosis stage II-IV was significantly higher than those with fibrosis stage 0-I (2.28 ± 0.68 m/s and 1.20 ± 0.24, respectively, p = 0.002). ARFI is able to differentiate significant from non significant liver fibrosis in patients with AIH. Our study suggests that this non-invasive method can be used for monitoring fibrosis progression in AIH.

Background. Despite advances in the understanding of the pathophysiological basis of autoimmune hepatitis (AIH), it is still difficult to delineate the mechanisms involved in progression from hepatic inflammation toward fibrosis. Our aim was to study serum concentrations of NO in AIH of different histological severity and possible effects of immunosuppressive therapy on NO production. Materials and methods. We studied serum NO metabolites (NOx) in 47 consecutive patients with AIH and in 28 age- and sex-matched controls. Results. Serum NOx concentrations were higher in AIH patients than in controls (10.3 (4.5–27.3 µmol/L) vs. 4.3 (1.6–14.3 µmol/L), p < 0.001). According to liver histology, median NOx concentrations were significantly higher in patients with severe interface hepatitis compared to patients with mild-moderate interface hepatitis (12.3 (4.5–27.3 µmol/L) vs. 9.3 (4.6–20.3 µmol/L), p = 0.029). Similarly, serum NOx concentrations were significantly higher in patients with advanced fibrosis than in those with early fibrosis (12.2 (4.6–27.3 µmol/L) vs. 9.3 (6.6–12.8 µmol/L), p = 0.018). NOx concentrations decreased in 16 AIH patients who were tested also after biochemical remission was achieved (12.6 (4.5–22.8 µmol/L) at baseline and 5.9 (2.8–10.5 µmol/L) after remission, p = 0.001). Conclusion. This study shows that serum NOx levels are associated with the histological severity of AIH. Hepatocyte inflammation and injury may activate hepatic stellate cells and kupffer cells, and the consequences may include release of NO, which ultimately promotes hepatic fibrosis. Immunosuppressive therapy inhibits this process and the production of NO.

There are scarce data regarding the impact of sphincterotome design on cannulation success. We aimed to compare two different 5.5 Fr standard sphincterotomes to determine initial cannulation success. Adult patients with naive papillae were enrolled in a prospective, randomized, crossover study. Two different 5.5 Fr sphincterotomes preloaded with guidewire (GW) were used in two groups with 140 patients included per group. A total of five papillary attempts and two pancreatic channel entries were allowed as maximum targets. In a case of more than two pancreatic entries, a double GW technique was attempted before crossover. If choledochal cannulation was not achieved within ten papillary attempts or more than four pancreatic entries despite crossover, access papillotomy was performed. Successful biliary cannulation was the primary outcome. Secondary outcomes were incidence of early complications and overall cannulation success. Higher initial cannulation success was achieved in group I compared with group II (88.5 vs. 77.1 %, p = 0.011). The crossover and double GW techniques reduced the need for precut from 11.7 to 5.3 %. The overall cannulation success including precut for failed cases was 99.2 % (group I) and 98.5 % (group II). Sphincterotome type, presence of crossover, and number of cannulation attempts were predictors of successful cannulation in multivariate analysis. There was a significant difference in cannulation success between two different 5.5 Fr sphincterotomes. The cannulation success was mainly governed by sphincterotome design which serves a proper spatial orientation during the procedure. The combined use of crossover and double GW techniques may substantially decrease precut necessity.