Autoimmune liver disease: Patients ask for a hepatological network and trained family doctors


Autoimmune liver disease is a health problem on which medicine is making great progress. “

Autoimmune diseases of the liver represent low prevalence chronic diseases, this entails a different attitude both from the training point of view and from the point of view of connection between general medicine and specialist medicine compared to those with higher prevalence.

These diseases are rapidly evolving in terms of scientific knowledge, patient management and therapies, also thanks to the growth of networks between specialists (including multidisciplinary ones) and shared databases. Drugs for rare diseases have a relative spending ceiling and this cannot be an obstacle to the spread of new drugs. In all this, patient associations are clamoring for a regional hepatological network to provide the sick with the best path of diagnosis and treatment. The Sicilian network can represent a model from which other regions can draw inspiration.

The multiregional webinar “THE PATH TO OBSTACLES OF THE LIVER DISEASE. FOCUS ON AUTOIMMUNE LIVER DISEASES ”organized by Motore Sanità with the non-conditioning sponsorship of ALFASIGMA and INTERCEPT PHARMACEUTICALS took stock of the situation by touching on various regional realities and highlighting strong points and needs to ensure the patient the best diagnosis and treatment path.

Adult autoimmune liver diseases are primary biliary cholangitis (PBC), autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC) and IgG4 mediated liver disease. They are considered relatively rare diseases, the prevalence of which in the European population is less than 1%, even if the epidemiological data available are certainly not accurate.

All these diseases, if not diagnosed and not treated adequately, progress in terms of clinical severity up to the stages of decompensated liver cirrhosis and, characteristic of PSC, to cholangiocarcinoma.

The point of the situation was made by Pierluigi ToniuttoDirector of the Hepatology and Liver Transplantation Unit ASUI Udine, who added:

“Although there are very sensitive and specific laboratory and diagnostic imaging tests for their diagnosis, autoimmune liver diseases are certainly under-diagnosed except in cases, common only in AIH, of clinical presentation such as acute hepatitis. This justifies the observation that although we now have effective therapies to treat all autoimmune liver diseases, except PSC, the percentage of liver transplants performed for these diseases has not decreased in the last 10 years, reaching 10% in Europe. The involvement and awareness of the general practitioner for the early diagnosis of autoimmune liver diseases is of primary importance, since in most cases these diseases remain asymptomatic for a long time and can only be suspected on the basis of impaired function tests. hepatic. Any alteration of liver function tests must be carefully evaluated and in the process of differential diagnosis it is also essential to consider the presence of an autoimmune liver disease “.

In Sicily, on the basis of the experience and success of the network set up for HCV, a network was activated at the end of 2020, the Sintesi PBC, Sicilian Network for Therapy, Epidemiology and Screening in Hepatology.

It was presented by Vincenza CalvarusoAssociate Professor, Gastroenterology Section, University of Palermo.

“The network is a fundamental tool for sharing strategies, for optimizing resources, for raising awareness of diseases, for obtaining data on epidemiology and for allowing more equity in access to diagnosis / treatment. The main objectives of the network are: to have clearer data on epidemiology, to identify response factors to first-line therapy, to evaluate the efficacy of second-line treatment, to identify new predictors of events, progression towards cirrhosis and eventual decompensation, evaluate comorbidities and vascular risk, standardize the management of symptoms in these patients. The network includes all patients with chronic liver diseases divided by etiology and the part dedicated to PBC is a web platform that records all patients diagnosed in the 13 Sicilian centers. 430 patients entered with basic characteristics similar to existing cohorts: 90% of women, 55 years of average age of diagnosis, 31.8% do not respond to UDCA treatment. The probability of progressing towards cirrhosis ranges from 3% in 5 years, 11.7% in 10 years and 27.2% in 15 years “.

According to Ignazio Grattagliano, President of SIMG Bari “We need a structured pathology network. It is true that Sicily has started a network that in some ways is working, but it must be implemented and many general practitioners who are still far from the network must connect. It is a reality that must be structured, because it is currently still based on voluntary mechanisms, otherwise there is a risk of losing it over time. Then it can be spread to other Italian regions, relocating it on the basis of different realities “.

Other topics addressed by Grattagliano: professional updating and technological innovation. “It cannot be of a wide range: the family doctor must be updated and receive information on the evolution of the disease. Being a low prevalence pathology, the family doctor, who is not an all-rounder, must be trained on the individual cases that follow, especially by dividing between those who have mild pathology and more aggressive pathology or patients destined for, or who have had, a transplant of liver. We can make great use of technological innovation and we hope that the PNRR will be able to incorporate digital innovation in a widespread way. To activate an initiative medicine that uses telemedicine, there is a need for trained personnel to manage patients through computer systems, not only medical personnel but also paramedical personnel, especially for when the territorial functional associations and community houses are ready. , where the presence of a general practitioner trained in the hepatological field of support for the classification and early diagnosis of patients will certainly be foreseen ”.

According to Pietro Invernizzi, Director of ASST San Gerardo di Monza, Professor at the Department of Gastroenterology of the Bicocca University of Milan, general practitioners have the great task of screening, of identifying the undeclared. “This is the main role to be attributed to general practitioners – explained Invernizzi -. Regarding the diagnosis, speaking of primary biliary cholangitis there are not only antimitochondrion, but there are also other antibodies and there are different laboratory techniques to test them. Costs vary and an idea developed in Lombardy to be disseminated is to imagine a PBC Reflex, for which an average cost will be defined for each request that is made to diagnose PBC. For general practitioners it is unthinkable that they know the details of all antibodies, but if they suspect PBC they could request antibodies to PBC, the cost of which would be low in the case of negative results and higher if they need further information “.

The patients were represented by EpaC and AMAF Onlus. Marco Bartoli, EpaC new drug access manager explained that patients and caregivers are very informed and interested in enriching their knowledge “for this reason Epac has implemented a whole series of activities to make them increasingly participatory and informed. Patients ask questions about the pathology, complications, symptom management and above all continually seek the opportunity to participate in trials, to obtain information on experimentation paths on new drugs or therapeutic options, for this reason it would be conceivable to create a tool that facilitates the recruitment or at least greater visibility of these trials “.

Another theme: the need to set up a regional hepatological network and provide more information: “Very often the PDTAs – he added Marco Bartoli – they are limited to very small areas and to fragment the hepatological world, for this reason it would be necessary to create a regional hepatological network in which the patient’s steps and centers of reference are identified.

Finally, we can create all the most beautiful paths but communication is the crux: it is difficult to find information and try to make it available to patients and centers of excellence “.

Davide Salvioni, President of AMAF ONLUS, Association of autoimmune liver diseases, focused on the early diagnosis and on the activities of the association. “In cases where liver function indices are not correct introducing an autoantibody evaluation could be an option that I would not rule out, this is part of a concept of a network between specialists and general practitioners in which the latter have a role important in identifying the warning signs of the disease.

Amaf has trained two expert patients to be able to speak at the discussion tables, we have an orientation line to be able to suggest to our associates the best treatment centers at national level, we offer moral support and soon we will open a desk dedicated to patients’ rights. Finally, since our birth, we have been fighting for recognition for the rare diseases of two pathologies, primary biliary cholangitis and autoimmune hepatitis, which although considered rare at European level are not at national level and with Uniamo we are collaborating to try to obtain the recognition”.

He closed the work table Franco Ripa, Head of Health and Social Health Planning. Vicar of the Piedmont Region Health and Welfare Directorate who explained: “Autoimmune liver diseases represent a health problem on which medicine is making great progress. To date, prevention measures are mainly of a generic and behavioral type. In this context, therefore, early diagnosis and the development of adequate management are fundamental to guide patients towards the most appropriate organizational settings and treatments, according to an integrated model between hospital and territory “.


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Autoimmune liver disease: Patients ask for a hepatological network and trained family doctors

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