Guidelines on the diagnosis and management of primary biliary cholangitis (2021) / 中华肝脏病杂志

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued a consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

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.

Novedades en enfermedades colestásicas / Latest advances in cholestatic diseases

Cholestatic liver diseases arise from impaired hepatobiliary production and excretion of bile. They have some common clinical manifestations and pathogenic features, but at the same time differences that require a special approach. Intrahepatic cholestasis of pregnancy and drug induced liver cholestasis are the most common reversible intrahepatic cholestasis. Primary biliary cirrhosis and primary sclerosing cholangitis are chronic cholestatic diseases. This review will be focused on these two types, in particular their clinical and therapeutic management and complications. While in recent years there has not been much change in the basic clinical approach of these diseases, every day we receive more information from both the basic and clinical science studies, which has enabled to develop new therapeutic lines and reject others that have not confirmed effectiveness.

Las enfermedades colestásicas se caracterizan por la disminución de la formación o excreción del flujo de bilis. Ellas tienen ciertas manifestaciones clínicas y mecanismos patogénicos comunes, pero a su vez diferencias que requieren un enfrentamiento no siempre similar. La colestasia intrahepática del embarazo(CIE) y la colestasia secundaria a fármacos son las más frecuentes dentro de las colestasias intrahepáticas reversibles. La cirrosis biliar primaria (CBP) y la colangitis esclerosante primaria (CEP), son las enfermedades colestásicas crónicas en que centramos esta revisión, en particular en los aspectos clínicos y de manejo terapéutico tanto de éstas, como de sus complicaciones. Si bien en los últimos años no ha habido un cambio significativo en el manejo fundamental de estas enfermedades, cada vez tenemos más información tanto en el área de ciencias básicas como en aspectos clínicos, lo que ha permitido ir desarrollando nuevas líneas terapéuticas y descartando otras que no han confirmado efectividad.

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia / Primary biliary cirrhosis: a thirteen years experience

Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

Tratamento MARS (Molecular Adsorbent Recirculating System) em pacientes com cirrose biliar primária: relato da primeira expectativa ambulatorial no Brasil / The MARS (Molecular Adsorbent Recirculating System): patient with primary biliary cirrhosis: first experience report in Brazil

O tratamento MARS (Molecular Adsorbent Recirculating System) permite a remoção de toxinas hepáticas ligadas à albumina. Dentre estas substâncias estão os ácidos biliares, que têm importante papel no prurido nas síndromes colestáticas. Relato do Caso: Descrição da primeira experiência brasileira ambulatorial de diálise MARS em paciente de 39 anos, sexo feminino, com diagnóstico de cirrose biliar primária e prurido recorrente após transplante de fígado e refratário às medidas clínicas, havendo também importante comprometimento na qualidade de vida. Após a realização de 2 sessões consecutivas do tratamento MARS, houve controle do prurido por pelo menos 7 meses. Procedimento bem tolerado e sem intercorrências Conclusão: O tratamento MARS é uma opção para pacientes com prurido intratável secundário a síndromes colestáticas e está associado à reduzida taxa de complicações

Cirrose biliar primária: revisão da literatura e relato de caso / Primary biliary cirrhosis: Literature reviews and case related

Cirrose biliar primária é uma entidade clínica rara e com poucos registros na literatura nacional. A etiologia é auto-imune e a incidência é predominante no sexo feminino. O quadro clínico compõe-se de diversos sinais e sintomas, além do quadro laboratorial específico (marcadores bioquímicos e imunológicos). Pode-se utilizar o diagnóstico por imagem, além do anatomopatológico. O tratamento clínico é controverso, sendo quase inevitável o transplante hepático após a instalação irreversível da insuficiência hepática. Após revisão bibliográfica os autores relatam um caso atendido no Hospital Universitário São Francisco de Assis

Osteopenia en cirrosis biliar primaria: estudio en 20 pacientes / Osteopenia in primary biliary cirrhosis: study in 20 patients

We studied 20 female patients, aged 51 ñ 13.6 years old, with the diagnosis of Primary Biliary Cirrhosis (PBC) to assess osteopenia, main involved sites and its relation to menopause, some parameters of mineral metabolism and the degree of histological liver involvement. The diagnosis of PBC was based in histological, clinical and laboratory features. Bone densitometry was measured with dual-photon densitometer and compared with values of a normal female population from the laboratory. When compared to controls, studied patients had a significantly lower lumbar spine bonedensity and total bone mineral content and a non significantly lower density in femoral neck and total body. Serum calcium, phosphorus, magnesium, PTH and urinary calcium/creatinine ratio were within normal limits. Lumbar spine density was no significantly lower in patients with more severe liver histological involvement and in postmenopausal women. No correlation was found between the duration of postmenopausal period and the degree of osteopenia. It is concluded that patients with PBC have a clear lumbar spine osteopenia and a lower total mineral content and that these parameters worsen in a non significant fashion in subjects along with liver histological involvement and with the length of post menopausal period