Important problems in the diagnosis and treatment of primary sclerosing cholangitis (based on the Russian consensus on diagnosis and treatment autoimmune hepatitis. Moscow, 2018).

The article is published based on the results of the Russian Consensus on the diagnosis and treatment of primary sclerosing cholangitis (PSC), discussed at the 44th annual Scientific Session of the CNIIG "Personalized Medicine in the Era of Standards" (March 1, 2018). The aim of the review is to highlight the current issues of classification of diagnosis and treatment of patients with PSC, which causes the greatest interest of specialists. The urgency of the problem is determined by the multivariate nature of the clinical manifestations, by often asymptomatic flow, severe prognosis, complexity of diagnosis and insufficient study of PSC, the natural course of which in some cases can be considered as a function with many variables in terms of the nature and speed of progression with numerous possible clinical outcomes. In addition to progression to portal hypertension, cirrhosis and its complications, PSC can be accompanied by clinical manifestations of obstructive jaundice, bacterial cholangitis, cholangiocarcinoma and colorectal cancer. Magnetic resonance cholangiography is the main method of radial diagnostics of PSC, which allows to obtain an image of bile ducts in an un-invasive way. The use of liver biopsy is best justified when there is a suspicion of small-diameter PSC, autoimmune cross-syndrome PSC-AIG, IgG4-sclerosing cholangitis. Currently, a drug registered to treat primary sclerosing cholangitis which can significantly change the course and prognosis of the disease does not exist. There is no unified view on the effectiveness and usefulness of ursodeoxycholic acid and its dosage in PSC. Early diagnosis and determination of the phenotype of PSC is of clinical importance. It allows to determine the tactics of treatment, detection and prevention of complications.

Important problems in the diagnosis and treatment of autoimmune hepatitis (based on the Russian consensus 2017).

The analysis of publications devoted to the Russian Consensus on the Diagnostic and Treatment of Autoimmune Hepatitis (AIH), which was considered at the 43rd annual Scientific Session of the CNIIG From Traditions to Innovation (March 4, 2017) is carried out. The presence of clear algorithms and recommendations for the diagnosis and treatment of AIH significantly help the doctor in real clinical practice, but do not exclude a personified approach to the patient.

[SCLEROUSIS CHOLANGITIS AT THE AUTOIMMUNE PANCREATITIS PATIENT].

The article contains the description of a clinical case of the patient P. of 59 years with autoimmune pancreatitis (AIP) associated with a sclerousis cholangitis (SC), and the analysis of literature on this problem. The patient was operated (cholecystenterostomy, enteroenterostomy) for pancreatic cancer. The pancreatic cancer was excluded. Overlap syndrome (primary biliary cirrhosis/autoimmune hepatitis), pseudotumorosis pancreatitis were excluded during the period of monitoring in hospitals of Moscow. In our clinic the diagnosis AIP is established on the basis of Mayo's criterion (HISORt: Histology, Imaging, Serology, Other organ involvement, Response to corticosteroid therapy). The increased level of immunoglobulin G (IgG) by 3 times (46,2 g/l, norm--6.58-18.37 g/l), IgG4 by 25 times (49.5 g/I, norm--0.33-2.01 g/l) was revealed. MRI of abdominal organs showed diffusion increase pancreas with a peripheral hypointensive rim, local stenosis of the Major pancreatic duct in the head pancreas. Immune histochemical study surgical biopsy of pancreas showed large number of IgG-positive plasma cells. Glucocorticosteroids (GCS, prednisolone 40 mg/d), ursodeoxycholic acid 1000 mg/d, creon 120000 ed/d promoted improvement of clinical picture AIP and decrease IgG4 (to 6.4 g/I). After 12 months IgG4 wasn't defined. Control MRI revealed dilatation small biliary ducts in 8, 5, 4 liver segments and thickening wall common bile duct (to 2 mm) at large distance. The peculiarity of the case--the 15-year follow-up of patients with AlP who had inappropriate surgery. Late diagnosis and delayed treatment GCS adversely affected the course of the disease, which led to the development of secondary biliary cirrhosis.

[FAMILIAL CLUSTERS OF HEPATITIS DELTA IN ENDEMIC REGION (REPUBLIC TYVA)].

AIM: Clinical and virological characteristic of hepatitis delta familial clusters in region of Russia that is endemic for this infection (Republic Tuva). MATERIALS AND METHODS: Total 383 patients with HBV/HDV coinfection and their family members (3 generations) were followed. Serum samples for HDV and HBV markers testing were available for 42 patients from 18 families. HBsAg, anti-HBc, HBeAg, anti-HBe and anti-HDV were tested using commercial ELISA tests; HDV RNA and HBV DNA were tested using in house nested PCR tests. RESULTS: 30 family (63 people) clusters were identified, in which close living persons have been infected with HDV and HBV. The biological material for determining of HDV and HBV markers has been available from 18 families (42 people belonging to 1-3 generations (parents and children, husband and wife, brother and sister). The mean age was 35 ± 14 years (10-58 years). Chronic hepatitis (CH) was in 30 (71.4%) patients, liver cirrhosis (LC)--in 10 (23.8%) and HCC was developed in 2 (4.8%) person on the background of long infections. The incidence of HBeAg was 14.3% (6/42), HBV DNA--19% (8/42); HDV RNA--35.7% (15/42). In 2 cases (mother) replication markers of both viruses were found, it contributed to the increased risk of infection in children. So HDV RNA was detected in the blood serum of their daughters (15 and 17), that does not exclude the possibility of vertical transmission. An illustration describes four families. CONCLUSION: The epidemic process of delta infection in the Republic of Tyva is characterized by intrafamilial infection of HBV and HDV.