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BACKGROUND: Cirrhosis is an important health problem characterized by a significant change in liver parenchyma. In animals, this can be reproduced by an experimental model of bile duct ligation (BDL). Melatonin (MLT) is a physiological hormone synthesized from serotonin that has been studied for its beneficial properties, including its antioxidant potential. AIM: To evaluate MLT's effects on oxidative stress, the inflammatory process, and DNA damage in an experimental model of secondary biliary cirrhosis. METHODS: Male Wistar rats were divided into 4 groups: Control (CO), CO + MLT, BDL, and BDL + MLT. MLT was administered (20 mg/kg) daily beginning on day 15 after biliary obstruction. On day 29 the animals were killed. Blood samples, liver tissue, and bone marrow were collected for further analysis. RESULTS: BDL caused changes in biochemical and histological parameters and markers of inflammatory process. Thiobarbituric acid (0.46 ± 0.01) reactive substance levels, superoxide dismutase activity (2.30 ± 0.07) and nitric oxide levels (2.48 ± 0.36) were significantly lower (P < 0.001) n the groups that received MLT. DNA damage was also lower (P < 0.001) in MLT-treated groups (171.6 ± 32.9) than the BDL-only group (295.5 ± 34.8). Tissue damage and the expression of nuclear factor kappa B, interleukin-1ß, Nrf2, NQO1 and Hsp70 were significantly lower in animals treated with MLT (P < 0.001). CONCLUSION: When administered to rats with BDL-induced secondary biliary cirrhosis, MLT effectively restored the evaluated parameters.

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ABSTRACT Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cirrhosis who developed a secondary Sjogren syndrome and vitamin A deficiency, which led to severe and unusual eye involvement with multiple and recurrent spontaneous corneal perforations. Corneal perforations in patients with primary biliary cirrhosis and secondary Sjogren syndrome are rare but devastating complications, in contrast to other eye clinical manifestations of the disease.

RESUMO A cirrose biliar primária é uma doença hepática autoimune progressiva rara que causa colestase crônica. 75% dos pacientes com Cirrose Biliar Primária desenvolvem Síndrome de Sjögren Secundária, e podem também desenvolver deficiência de vitamina A. Aqui, relatamos um paciente com Cirrose Biliar Primária que desenvolveu Síndrome de Sjögren Secundária e deficiência de vitamina A, levando a envolvimento ocular grave e incomum com perfurações espontâneas múltiplas e recorrentes da córnea. Perfurações da córnea em pacientes com Cirrose Biliar Primária e Síndrome de Sjögren Secundária são complicações raras, mas devastadoras, em contraste com outras manifestações clínicas oculares da doença.

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RESUMEN Introducción: La cirrosis hepática constituye la etapa final de muchas enfermedades del hígado. Objetivo: Determinar las características epidemiológicas y clínicas de pacientes con cirrosis hepática. Métodos: Se realizó una investigación descriptiva, retrospectiva, con datos de archivo, de 57 pacientes cirróticos. Se excluyeron aquellos con datos insuficientes en la historia clínica. Las variables analizadas fueron: edad, sexo, etiología, modo de diagnóstico, comorbilidades, manifestaciones endoscópicas de la hipertensión portal, complicaciones, estadios de la enfermedad según D'Amico y clasificación de Child-Pugh. Para el análisis de los resultados se emplearon técnicas de la estadística descriptiva. Resultados: Hubo 19 pacientes (33,3 %) en el grupo de edad entre 60 y 69 años; 31 (54,4 %) mujeres y 26 (45,6 %) hombres. El virus de la hepatitis C fue encontrado en 21 pacientes (36,8 %). El 96,4 % de los enfermos se diagnosticaron mediante ecografía abdominal; 12 (21,1 %) presentaron ascitis y 38 (66,6 %) manifestaciones endoscópicas de hipertensión portal. En estadio 4 de D'Amico estaban 20 (35 %) enfermos y 26 (45,6 %) en estadio de Child-Pugh A; 24 (42,1 %) en Child-Pugh B y 7 (12,3 %) en Child-Pugh C. Conclusiones: La cirrosis hepática predomina en la séptima década de la vida, en el sexo femenino. Prevalece el ultrasonido abdominal como modo de diagnóstico. Las causas más frecuentes son el virus de hepatitis C y el alcoholismo. La ascitis es la complicación que más se presenta. La mayoría de los pacientes muestran signos de hipertensión portal. Predominan las formas no compensadas de la enfermedad.

ABSTRACT Introduction: Liver cirrhosis is the end stage of many liver diseases. Objective: To determine the epidemiological and clinical characteristics of patients with liver cirrhosis. Results: There were 19 patients (33.3%) in the age group between 60 and 69 years; 31 (54,4 %) women and 26 (45,6 %) men. Hepatitis C virus was found in 21 patients (36,8 %). 96,4 % of the patients were diagnosed by abdominal ultrasound; 12 (21,1 %) presented ascites and 38 (66,6 %) endoscopic manifestations of portal hypertension. In D'Amico stage 4 there were 20 (35 %) patients and 26 (45,6 %) in Child-Pugh A stage; 24 (42,1 %) in Child-Pugh B and 7 (12,3 %) in Child-Pugh C. Conclusions: Liver cirrhosis predominates in the seventh decade of life, in females. Abdominal ultrasound prevails as a diagnostic mode. The most common causes are hepatitis C virus and alcoholism. Ascites is the most common complication. Most patients present with signs of portal hypertension. Uncompensated forms of the disease predominate.

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BACKGROUND: Pruritus is a major health-related quality-of-life burden in progressive familial intrahepatic cholestasis (PFIC) and other childhood cholestatic liver diseases. Several nontransplant surgical techniques were developed in an attempt to ameliorate symptoms and slow disease progression. Very few case-series have been published on a particular intervention, ileal exclusion (IE), which has been considered to be inferior to the other approaches. METHODS: We conducted a single-center retrospective chart-review case-series of patients submitted to IE as the first-line surgical treatment at our institution from 1995 to 2018. The primary goal was pruritus relief, followed by survival with the native liver and improvement in biochemical parameters. RESULTS: Eleven patients were submitted to IE, with a mean follow-up of 60 months. Complete resolution or significant reduction of pruritus was obtained in 72.7% (n = 8) of patients. One patient (9.1%) had a major postoperative complication that required surgery. No other morbidities were reported. Two cases progressed to end-stage liver disease (ESLD) within the short-term and one year after surgery. CONCLUSIONS: This case series study shows that IE provided excellent results in pruritus control and permitted survival with the native liver. We believe IE is a safe procedure, with few associated morbidities, and should be considered more often as primary surgical treatment for PFIC and other cholestasis. LEVEL OF EVIDENCE: IV.

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ATP-binding cassette (ABC) subfamily B member 4 (ABCB4), also known as multidrug resistance protein 3 (MDR3), encoded by ABCB4, is involved in biliary phospholipid secretion, protecting hepatobiliary system from deleterious detergent and lithogenic properties of the bile. ABCB4 mutations altering canalicular ABCB4 protein function and expression may have variable clinical presentation and predispose to several human liver diseases. Well-established phenotypes of ABCB4 deficit are: progressive familial intrahepatic cholestasis type 3, gallbladder disease 1 (syn. low phospholipid associated cholelithiasis syndrome), high ɣ-glutamyl transferase intrahepatic cholestasis of pregnancy, chronic cholangiopathy, and adult biliary fibrosis/cirrhosis. Moreover, ABCB4 aberrations may be involved in some cases of drug induced cholestasis, transient neonatal cholestasis, and parenteral nutrition-associated liver disease. Recently, genome-wide association studies have documented occurrence of malignant tumours, predominantly hepatobiliary malignancies, in patients with ABCB4/MDR3 deficit. The patient's age at the time of the first presentation of cholestatic disease, as well as the severity of liver disorder and response to treatment are related to the ABCB4 allelic status. Mutational analysis of ABCB4 in patients and their families should be considered in all individuals with cholestasis of unknown aetiology, regardless of age and/or time of onset of the first symptoms.

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OBJECTIVES: In cirrhotic children, infection events and sepsis are more frequent and more severe due to immune dysfunction. The objectives of the current study were therefore to develop an experimental model of infection and sepsis in cirrhotic weaning growing rats, by the use of bile duct ligation (BDL) and cecal ligation and puncture (CLP). Additionally, the correlation of the clinico-histopathological data and serial cytokine levels in septic cirrhotic and non-cirrhotic animals was studied. METHODS: Young Wistar rats of age 21 days and of weight between 70-90 g were divided into 12 groups according to the surgical procedure performed: sham (sacrificed after 2 or 4 weeks), BDL (sacrificed after 2 or 4 weeks), CLP (2- or 4-week old animals sacrificed after 12 or 24 hours), BDL+CLP (2- or 4-week old animals sacrificed after 12 hours). Histopathological studies and determination of serum levels of cytokines IL-1 beta, IL-10, and TNF-alpha, for studies of systemic infection, were performed. Murine sepsis scores (MSS) based on the clinical aspects just before euthanasia were also included. RESULTS: A transitory increase in IL-1, IL-10, and TNF-alpha levels was observed, with different patterns according to the groups. Two-hit groups tended to present with higher values of serum cytokines and histopathological scores than their septic non-cirrhotic counterparts. There was a correlation between mortality rate and MSS (p<0.0001). CONCLUSION: The model is feasible and may be utilized in studies on liver cirrhosis and infection in growing animals.

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Primary biliary cholangitis (PBC) is an autoimmune liver disease associated with altered lipoprotein metabolism, mainly cholesterol. Hypercholesterolaemia, a major modifiable risk factor for cardiovascular disease in the general population, occurs in 75%-95% of individuals with PBC. The impact of hypercholesterolaemia on cardiovascular risk in PBC, however, is controversial. Previous data have shown that hypercholesterolaemia in PBC is not always associated with an increase in cardiovascular events. However, patients with PBC with cardiovascular risk factors may still warrant cholesterol-lowering therapy. Treatment of hypercholesterolaemia in PBC poses unique challenges among primary care providers due to concerns of hepatotoxicity associated with cholesterol-lowering medications. This review summarises the current understanding of the pathophysiology of hypercholesterolaemia in PBC and its pertinent cardiovascular risk. We will also discuss indications for treatment and the efficacy and safety of available agents for hypercholesterolaemia in PBC.

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BACKGROUND: Biliary cirrhosis is associated with hepatopulmonary syndrome (HPS), which is related to increased posttransplant morbidity and mortality. AIMS: This study aims to analyze the pathophysiology of biliary cirrhosis and the onset of HPS. METHODS: Twenty-one-day-old Wistar rats were subjected to common bile duct ligation and were allocated to two groups: group A (killed 2, 3, 4, 5, or 6 weeks after biliary obstruction) and group B (subjected to biliodigestive anastomosis 2, 3, 4, 5, or 6 weeks after the first procedure and killed 3 weeks later). At the killing, arterial blood was collected for the analyses, and samples from the liver and lungs were collected for histologic and molecular analyses. The gasometric parameters as well as the expression levels of ET-1, eNOS, and NOS genes in the lung tissue were evaluated. RESULTS: From a total of 42 blood samples, 15 showed hypoxemia (pO2 < 85 mmHg) and 17 showed an increased oxygen gradient [p (A-a) O2 > 18 mmHg]. The liver histology revealed increased ductular proliferation after common bile duct ligation, and reconstruction of bile flow promoted decreased ductular proliferation 5 and 6 weeks post-common bile duct ligation. Pulmonary alterations consisted of decreased parenchymal airspace and increased medial wall thickness. Biliary desobstruction promoted transitory improvements 5 weeks after biliary obstruction (increased parenchymal airspace and decreased MWT-p = 0.003 and p = 0.004, respectively) as well as increased endothelin expression levels (p = 0.009). CONCLUSIONS: The present model showed lung tissue alterations promoted by biliary obstruction. The biliodigestive anastomosis had no clear direct effects on these alterations.

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INTRODUCTION: Several groups have reported associations of primary biliary cholangitis with other autoimmune entities, particularly Sjögren's syndrome and hypothyroidism. Its prevalence and characteristics in Mexican patients is unknown. AIM: To determine the frequency and characteristics of autoimmune diseases in a Mexican cohort of patients with primary biliary cholangitis. MATERIALS AND METHODS: The medical records of patients that presented with primary biliary cholangitis within the time frame of 2005 and 2012 were reviewed and assessed for other autoimmune diseases. RESULTS: Seventy-eight patients, 75 women and 3 men, were included. Their mean age was 55.8 years. Seventy-three cases had positive antimitochondrial antibodies (94.8%) and disease was confirmed in 5 through liver biopsy. Five patients (8%) had anti-smooth muscle antibodies and 55/78 (70.5%) had antinuclear antibodies by indirect immunofluorescence. Forty-nine patients (62.8%) were positive for an autoimmune disease other than primary biliary cholangitis. Among those, 20 patients had one associated disease, 14 had 2, and 15 patients had 3 concomitant diseases. They included: Sjögren's syndrome in 23/78 patients (29.5%), dysthyroidism in 21/78 cases (26.9%), Raynaud syndrome in 11/78 (14.1%), CREST syndrome in 9/78 patients (11.4%), rheumatoid arthritis in 6/78 patients (7.7%), vitiligo in 5/78 (6.4%), scleroderma in 4/78 patients (5.1%), and other diseases in 8 patients. In 12/78 patients (15.4%), there was a documented family background of autoimmune disease. CONCLUSIONS: The presence of autoimmune associations in our cohort was frequent, and similar in characteristics to the information reported by other groups. The clinical implications of those findings remain to be determined.

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El síndrome de Reynolds, se define como cirrosis biliar primaria en pacientes con esclerodermia; este síndrome debe ser sospechado en aquellos pacientes que desarrollen un patrón colestásico. Se reporta una paciente con antecedente de esclerodermia que se presenta con ictericia, a quien se le confirma con estudios inmunológicos y biopsia hepática, el diagnóstico de cirrosis biliar prima­ ria (ahora se denomina colangitis biliar primaria). Se ordena ácido ursodesoxicólico 15mg/día.

Reynolds syndrome is defined as primary biliary cirrhosis in patients with scleroderma; this syndro­me should be suspected in those patients who develop a cholesteric pattern. We report a patient with scleroderma who presented with jaundice. After immunological and liver biopsy, a diagnosis of Primary Biliary Cholangiopathy (new name) was confirmed. Ursodeoxycholic acid 15mg / day was prescribed

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BACKGROUND: Autoimmune hepatitis (AIH) with characteristics of primary biliary cholangitis (PBC) is known as overlap syndrome. Its prevalence and prognosis have not yet been determined comparatively with AIH. METHODS: A retrospective cohort study was conducted comparing patients diagnosed with AIH and AIH-PBC overlap syndrome, followed-up for seven years in a university hospital in Colombia, until 31 December 2016. RESULTS: A total of 210 patients were included (195 women, mean age 48.5years). Of these, 32 (15.2%) had AIH-PBC overlap syndrome. At diagnosis, no significant differences were found by demographic profile, positive autoantibodies (ANA, ASMA), except AMA (81.2% vs 3.9%, P<.001), and histological grade of fibrosis. The most frequent clinical presentations were nonspecific symptoms in AIH-PBC and acute hepatitis in AIH. Although there were no significant differences, AIH showed a greater biochemical response to immunosuppressive management (87.3% vs 74.2%, P=.061) and a greater number of relapses in those who achieved partial or complete remission during treatment (12.4% vs 7.63%; P=.727). Patients with AIH-PBC had greater progression to cirrhosis (22.2% vs 13.1%, P=.038), even in those who achieved partial or complete biochemical remission without relapse, with greater indication of orthotopic liver transplantation (P=.009), but not retransplantation (P=.183); there were no differences in mortality. CONCLUSIONS: AIH-PBC overlap syndrome accounts for a significant proportion of patients with AIH, with greater progression to cirrhosis, indication of liver transplantation and possibly retransplantation. This higher risk of adverse outcomes suggests closer monitoring, probably with follow-up until confirmed histopathological remission.

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BACKGROUND/PURPOSE: Biliary atresia and other liver biliary obstructions are relevant conditions in pediatric surgery due to their progression to biliary cirrhosis and indication for liver transplantation. It is known that the period during which biliary obstruction persists determines the development of cirrhosis and its reversibility after a biliary drainage procedure. However, no time or histological markers of biliary cirrhosis reversibility have been established. MATERIALS AND METHODS: One hundred and twenty-nine young Wistar rats underwent surgery for ligation of the common bile duct and were maintained until 8weeks. A part of these animals was submitted to biliary drainage surgery at 2, 3, 4, 5, or 6weeks after the initial procedure. After cyst formation at the site of obstruction, cyst-jejunal anastomosis was performed to restore bile flow. After biliary obstruction and drainage, liver samples were collected for histological and molecular analysis of the genes responsible for collagen deposition and fibrosis. RESULTS: The mortality rates were 39.8% and 56.7% after the first and second procedures, respectively. Ductular proliferation (p=0.001) and collagen deposition increased according to the period under obstruction (p=0.0001), and both alterations were partially reduced after biliary drainage. There were no significant differences in the values of desmin and α-actin according to the period during which the animal remained with biliary obstruction (p=0.09 and p=0.3, respectively), although increased values of transforming growth factor beta 1 (TGFß1) occurred after 8weeks (p=0.000). Desmin levels decreased, and α-actin and TGFß1 levels increased according to the period under obstruction. The molecular alterations were partially reversed after biliary drainage. CONCLUSIONS: The histologic and molecular changes in the liver parenchyma promoted by biliary obstruction in the young animal can be partially reversed by a biliary drainage procedure.

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BACKGROUND: Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean countries. OBJECTIVES: The aim of our study was to investigate the clinical patterns, laboratory findings, and immunologic features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. METHODS: A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba, from May 2012 to April 2016. Clinical, immunologic, and histologic features of autoimmune hepatitis (AIH), primary biliary cirrhosis, AIH/primary biliary cirrhosis overlap syndrome, autoimmune cholangiopathy, and primary sclerosing cholangitis were recorded. Response to therapy was assessed by serum alanine aminotransferase and bilirubin levels at 3, 6, 12, and 24 months after treatment initiation. RESULTS: Of the 106 patients included in the study, 85.5% were women. The median age at presentation was 47 years. AIH was the most common AILD and was diagnosed in 60 patients (56.6%), 55 of whom had type 1 AIH. Primary biliary cirrhosis was diagnosed in 22 patients (20.7%), overlap syndrome in 16 patients (15%), autoimmune cholangiopathy in 5 patients (4.71%), and PSC in 3 patients (2.8%). Most patients were symptomatic; 48 patients (45.2%) presented with liver cirrhosis, 14.5% of whom had decompensated cirrhosis. Follow-up of treatment was between 6 and 24 months. Prednisone monotherapy was used in 22 AIH patients (36.6%) and a combination of prednisone and azathioprine was used in 28 (46.6%) AIH patients. Response to treatment was seen in 41 AIH patients (68.3%), 33 of whom (55%) had a complete response and 8 of whom (24.2%) relapsed after 12 months of maintenance therapy. No or incomplete response to treatment was seen in 18 patients (30%). In 46 patients with autoimmune cholestasis, ursodeoxycholic acid was used as monotherapy in 25 patients (54.3%). CONCLUSIONS: The clinical profile of AILD in a sample of the Cuban population is similar to that reported in South areas (Developing countries). AIH was more frequent than PBC, and usually presented with advanced liver disease that responded poorly to treatment.

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Resumen La esclerosis sistèmica (esclerodermia) es la enfermedad reumática autoinmunitaria asociada más comúnmente con cirrosis biliar primaria. La relación entre la forma cutánea limitada de esclerosis sistémica y cirrosis biliar primaria se describió en el decenio de 1970. Se comunica el caso de una paciente de 65 años de edad con esclerosis sistémica cutánea limitada de larga evolución, positiva a anticuerpos contra centròmero y mitocondria. La biopsia hepática confirmó cirrosis biliar primaria en fase portal (estadio 1).

Abstract Systemic sclerosis (scleroderma) is an autoimmune rheumatic disease most commonly associated with primary biliary cirrhosis. The relationship between limited cutaneous systemic sclerosis and primary biliary cirrhosis was first described in the early 1970's decade. The case of 65 year-old woman with limited cutaneous systemic sclerosis and primary biliary cirrhosis seropositive for anticentromere and anti-mitochondrial antibodies is reported. Liver biopsy confirmed primary biliary cirrhosis in stage 1.

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La sobrevida de los pacientes postrasplante hepático supera el 90% al año y el 75% a los 5 años. Entender las causas de pérdida del injerto, o inclusive la muerte del paciente, es esencial para mejorar aún más los resultados a largo plazo. La evaluación de las biopsias hepáticas tiene un papel importante en la explicación y manejo de la disfunción del injerto de hígado, que ocurre después del primer año del trasplante. La interpretación de estas biopsias puede ser muy difícil, en especial por la alta incidencia de enfermedades recurrentes que pueden mostrar un cuadro clínico y unas características histopatológicas que semejan varias condiciones, especialmente cuando el rechazo agudo o crónico pueden sobreponerse a una patología ya existente o presentarse de manera simultánea y contribuir a la disfunción tardía del injerto, por lo que el análisis de la biopsia puede ayudar a determinar el componente principal de la lesión. Es indispensable la correlación clínico patológica, teniendo en cuenta la enfermedad original, el tipo de inmunosupresión, las pruebas de función hepática, las serologías virales, los autoanticuerpos y los hallazgos radiológicos. En este artículo comentaré las patologías más frecuentes y las que causan más problemas en su diagnóstico durante el período postrasplante tardío

One year survival rates of liver transplant patients exceed 90% while five year survival rates exceed 75%. Understanding the causes of graft losses and patient deaths is essential for further improvement of long-term results. Evaluation of liver biopsies has an important role in explaining liver graft dysfunction that occurs more than one year after transplantation, and thus is key for post-transplant patient management. The interpretation of these biopsies can be very difficult especially because of the high incidence of recurrent diseases that sometimes have clinical and histopathological features that resemble various other conditions. This is especially true for acute and chronic rejection which can overwhelm an existing condition and which can develop simultaneously with other conditions that contribute to late graft dysfunction. Analysis of the biopsy can help determine the main component of a lesion. Clinical findings must be correlated to pathological findings, and the correlation must take into account the original disease, the type of immunosuppression, liver function tests, viral serology, autoantibodies and radiological findings. In this article I will discuss the most common diseases and those that cause the most problems for diagnosis during the late post-transplant period

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Introducción: El Síndrome de Reynolds es la asociación de esclerosis sistémica (SSC) con cirrosis biliar primaria (CBP). Descrito en 1934 por Milbradt y Reynolds en 1976 describió seis casos. Caso: Presentamos tres casos de mujeres atendidas en el Servicio de Reumatologìa del Hospital Carlos Andrade Marín, con patología hepática autoinmune y signos de esclerosis sistémica. El diagnóstico fue confirmado mediante biopsia hepática en dos de ellas y la prueba de fibromax en la restante. Discusión: Reconocer el Síndrome de Reynolds permite el diagnóstico temprano de cirrosis biliar primaria en pacientes con esclerosis sistémica y sospechar esta entidad en quienes padecen enfermedad hepática colestásica autoinmune que, muchas veces, coexisten en forma silenciosa. El diagnóstico oportuno permite intervenciones terapéuticas precoces que podrían mejorar el pronóstico de esta asociación.

Introduction: The Reynolds' syndrome is the combination of systemic sclerosis (SSC) and primary biliary cirrhosis (PBC). It was first described in 1934 by Milbradt. Reynolds in 1976 presented six cases in two of them and fibromax-test in another. Case report: We present three cases of three women treated in the Rheumatology department at Carlos Andrade Marin hosppital who had autoinmune liver disease confirmed by biopsy. Discusion: Recognizing Reynolds' Syndrome allow us to make earlier diagnosis. This autoimmune cholestatic liver disease often remains silent, so that their identification is a valuable diagnostic tool leading to therapeutic interventions.

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There are many autoimmune diseases associated with primary biliary cholangitis (PBC), known as primary biliary cirrhosis; however, the association between PBC and warm autoimmune hemolytic anemia (wAIHA) has rarely been reported. It is documented that hemolysis is present in over 50% of the patients with chronic liver disease, regardless of the etiologies. Due to the clear and frequent relationship between PBC and many autoimmune diseases, it is reasonable to suppose that wAIHA may be another autoimmune disorder seen in association with PBC. Here we reported a 53-year-old female patient diagnosed with wAIHA associated with PBC.

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ABSTRACT In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

RESUMO Para definir as recomendações baseadas em evidências científicas sobre o diagnóstico e tratamento das doenças autoimnus do fígado, a Sociedade Brasileira de Hepatologia organizou em Outubro de 2014, encontro monotemático em São Paulo. Um Comitê organizador de sete investigadores foi selecionado pela Diretoria da Sociedade para organizar a agenda científica, assim como para selecionar vinte debatedores para fazer uma revisão sistemática e apresentar tópicos relacionados à hepatite autoimune, colangite esclerosante primária, cirrose biliar primária e suas síndromes de superposição (overlap). O texto inicial do submetidoo a apreciação e aprovação da Sociedade Brasileira de Hepatologia através de consulta a todos associados através da home page da Sociedade, O trabalho apresentado representa a versão final do trabalho original, devidamente revisado e organizado em tópicos, segundo as recomendações da Sociedade Brasileira de Hepatologia.

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