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


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Abstract

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

About the authors

E V Vinnitskaya

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

Email: e.vinnitskaya@mknc.ru
д.м.н., зав. отд. гепатологии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

S R Abdulkhakov

Kazan Federal University

к.м.н., с.н.с., ФГАОУ ВО КФУ Kazan, Russia

D T Abdurakhmanov

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

д.м.н., проф., ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

R B Alikhanov

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

к.м.н., зав. отд-нием хирургии печени и поджелудочной железы ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

I G Bakulin

I.I. Mechnikov North-Western State Medical University of the Ministry of Health of the Russian Federation

д.м.н., проф., ФГБОУ ВО «СЗГМУ им. И.И. Мечникова» Saint Petersburg, Russia

E A Belousova

M.F. Vladimirsky Moscow Regional Research Clinical Institute

д.м.н., проф., зав. каф. гастроэнтерологии ГБУЗ МО «МОНИКИ им. М.Ф. Владимирского» Moscow, Russia

A O Bueverov

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

д.м.н., профессор, ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

E Z Burnevitch

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

к.м.н., ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

M G Efanov

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., проф., руководитель отд. гепатобилиарной хирургии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

E Yu Eremina

Medical Institut of the N.P. Ogarev Mordovia State University

д.м.н., проф., зав. каф. пропедевтики внутренних болезней Медицинского института ФГБОУ ВО «НИМГУ им. Н.П. Огарева» Saransk, Russia

T M Ignatova

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

д.м.н., ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

L Yu Ilchenko

N.I. Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation

д.м.н., проф., ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Moscow, Russia

G G Karmazanovsky

A.V. Vishnevsky Institute of Surgery of the Ministry of Health of the Russian Federation

д.м.н., проф., ФГБУ «Институт хирургии им. А.В. Вишневского» Moscow, Russia

O V Knyazev

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., зав. отд-нием воспалительных заболеваний кишечника ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

Yu V Kulezneva

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., зав. отд. лучевых методов диагностики и лечения ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

T N Lopatkina

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

к.м.н., доц., ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

T P Nekrasova

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

к.м.н., доц. каф. патологической анатомии ФБГОУ ВО «Первый МГМУ им. И.М. Сеченова» Moscow, Russia

I G Nikitin

N.I. Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation

д.м.н., проф., ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Moscow, Russia

V V Pavlenko

Stavropol State Medical University of the Ministry of Health of the Russian Federation

д.м.н., проф., зав. каф. терапии ФГБОУ ВО СтГМУ Stavropol, Russia

A I Parfenov

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., проф., зав. отд. патологии кишечника ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

S D Podymova

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., проф., в.н.с. отд. гепатологии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

K L Raichelson

St Petersburg State University

д.м.н., проф. научно-клинического и образовательного центра гастроэнтерологии и гепатологии Института высоких медицинских технологий ФГБОУ ВО СПбГУ Saint Petersburg, Russia

A R Reisis

Central Research Institute of Epidemiology of the Federal Service on Customers

д.м.н., проф. научно-консультативного клинико-диагностического центра ЦНИИ Эпидемиологии Moscow, Russia

R G Sayfutdinov

Kazan State Medical Academy - the Branch of Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation

д.м.н., проф., зав. каф. КГМА - филиал ФГБОУ ДПО РМАНПО Kazan, Russia

E V Skazyvaeva

I.I. Mechnikov North-Western State Medical University of the Ministry of Health of the Russian Federation

к.м.н., врач-терапевт и гастроэнтеролог ФГБОУ ВО «СЗГМУ им. И.И. Мечникова» Saint Petersburg, Russia

V E Syutkin

N.V. Sklifosovsky Research Institute of Emergency Medical Care of the Department of Health of Moscow

д.м.н., проф., ГБУЗ «НИИ СП им. Н.В. Склифосовского» ДЗМ Moscow, Russia

S G Khomeriki

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., проф., зав. лаб. патоморфологии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

T Yu Haimenova

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

д.м.н., проф., зав. лаб. патоморфологии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

Yu G Sandler

A.S. Loginov Moscow Clinical Research and Practical Center of the Department of Health of Moscow

к.м.н., с.н.с. научно-исследовательского отд. гепатологии ГБУЗ «МКНЦ им. А.С. Логинова» ДЗМ Moscow, Russia

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