Registry of patients with autoimmune liver diseases in St. Petersburg: clinical and epidemiological data

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Abstract

BACKGROUND: The problem of autoimmune liver diseases, which include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and their overlap syndromes is an urgent problem of modern medicine. This is associated with an increase in the incidence and prevalence of these nosologies in different regions of the world. Absence of an established etiological factor, prolonged asymptomatic or low-symptomatic course, lack of pathognomonic clinical picture, imperfect diagnostic criteria, insufficient awareness of physicians lead to late diagnosis of autoimmune liver diseases and, accordingly, untimely initiation of treatment, which often reduces its effectiveness, affecting the further prognosis and survival rate for this group of patients. Patients with autoimmune liver diseases require long-term, often lifelong therapy and dispensary observation. Epidemiological registries along with autoimmune liver diseases registries, including various nosologies of autoimmune liver diseases, form an important database on the incidence, clinical features, development and course of the disease, variants of therapy and response to therapy, presence of extrahepatic manifestations as well as allow analysis of all data in dynamics.

AIM: To analize data on various nosologies of autoimmune liver diseases, demographics, clinical symptoms, timing of the first symptoms, timing of diagnosis, and the nature of extrahepatic manifestations.

MATERIALS AND METHODS: This article presents data from the autoimmune liver diseases Register of the North-West Hepatology Centre (North-West Autoimmune Liver Diseases Register) of the North-Western State Medical University named after I.I. Mechnikov.

RESULTS: The study obtained data on the nosological structure, demographic characteristics, timing of diagnosis verification, clinical symptoms and extrahepatic manifestations of autoimmune liver diseases.

CONCLUSIONS: Establishing registers of autoimmune liver diseases, providing information about patients with these diseases, will help to assess the effectiveness of examination and treatment methods, factors affecting the course and prognosis of the disease. Analysis of the data obtained will help to develop screening programs, diagnostic algorithms and differential diagnostics of autoimmune liver diseases, which, in turn, will facilitate the diagnostic process, shorten the time of diagnosis, make it possible to start therapy earlier and, consequently, improve the prognosis and survival rate for this group of patients.

About the authors

Igor G. Bakulin

North-Western State Medical University named after I.I. Mechnikov

Email: igbakulin@yandex.ru
ORCID iD: 0000-0002-6151-2021
SPIN-code: 5283-2032

MD, Dr. Sci. (Med.), Professor

Russian Federation, Saint Petersburg

Svetlana A. Burlakova

North-Western State Medical University named after I.I. Mechnikov

Email: burlakova20012010@gmail.com
ORCID iD: 0000-0002-6659-2622
SPIN-code: 3550-3719
Russian Federation, Saint Petersburg

Ekaterina V. Skazyvaeva

North-Western State Medical University named after I.I. Mechnikov

Email: skazyvaeva@yandex.ru
ORCID iD: 0000-0002-8563-6870
SPIN-code: 5882-1152

MD, Cand. Sci. (Med.), Assistant Professor

Russian Federation, Saint Petersburg

Maria I. Skalinskaya

North-Western State Medical University named after I.I. Mechnikov

Author for correspondence.
Email: mskalinskaya@yahoo.com
ORCID iD: 0000-0003-0769-8176
SPIN-code: 2596-5555

MD, Cand. Sci. (Med.), Assistant Professor

Russian Federation, Saint Petersburg

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Supplementary files

Supplementary Files
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2. Fig. 1. Nosological structure of the register. AIH — autoimmune hepatitis; PBC — primary biliary cholangitis; PSC — primary sclerosing cholangitis; DIAIH — drug-induced autoimmune hepatitis

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3. Fig. 2. Distribution of patients by age

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4. Fig. 3. Distribution of patients by gender

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5. Fig. 4. Distribution patients by gender depending on nosology. AIH — autoimmune hepatitis; PBC — primary biliary cholangitis; PSC — primary sclerosing cholangitis; DIAIH — drug-induced autoimmune hepatitis

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6. Fig. 5. Age of patients at the onset of symptoms of autoimmune liver diseases

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7. Fig. 6. Age of patients at diagnosis of autoimmune liver diseases

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8. Fig. 7. Time from onset of symptoms to diagnosis of autoimmune liver disease

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9. Fig. 8. Time from onset of symptoms to diagnosis of autoimmune liver disease depending on nosology. AIH — autoimmune hepatitis; PBC — primary biliary cholangitis; PSC — primary sclerosing cholangitis; DIAIH — drug-induced autoimmune hepatitis

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10. Fig. 9. Proportion of the patients with liver cirrhosis at the time of diagnosis of autoimmune liver diseases

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11. Fig. 10. Proportion of the patients with liver cirrhosis at the time of diagnosis of autoimmune liver diseases depending on nosology

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