| Autor: |
Vinnitskaya EV; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Abdulkhakov SR; Kazan Federal University, Kazan, Russia., Abdurakhmanov DT; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Alikhanov RB; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Bakulin IG; I.I. Mechnikov North-Western State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia., Belousova EA; M.F. Vladimirsky Moscow Regional Research Clinical Institute, Moscow, Russia., Bueverov AO; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Burnevitch EZ; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Efanov MG; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Eremina EY; Medical Institut of the N.P. Ogarev Mordovia State University, Saransk, Russia., Ignatova ТМ; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Ilchenko LY; N.I. Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia., Karmazanovsky GG; A.V. Vishnevsky Institute of Surgery of the Ministry of Health of the Russian Federation, Moscow, Russia., Knyazev OV; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Kulezneva YV; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Lopatkina TN; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Nekrasova TP; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia., Nikitin IG; N.I. Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia., Pavlenko VV; Stavropol State Medical University of the Ministry of Health of the Russian Federation, Stavropol, Russia., Parfenov AI; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Podymova SD; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Raichelson KL; St Petersburg State University, Saint Petersburg, Russia., Reisis AR; Central Research Institute of Epidemiology of the Federal Service on Customers, Moscow, Russia., Sayfutdinov RG; Kazan State Medical Academy - the Branch of Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation, Moscow, Russia., Skazyvaeva EV; I.I. Mechnikov North-Western State Medical University of the Ministry of Health of the Russian Federation, Saint Petersburg, Russia., Syutkin VE; N.V. Sklifosovsky Research Institute of Emergency Medical Care of the Department of Health of Moscow, Moscow, Russia., Khomeriki SG; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Haimenova TY; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia., Sandler YG; A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow, Moscow, Russia. |
| Abstrakt: |
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. |
