Colangitis biliar primaria: experiencia de cinco años en el Hospital Clínico de la Universidad de Chile / Primary biliary cholangitis. Experience in 179 patients

BACKGROUND: Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic disease, which can progress to cirrhosis. It mainly affects middle-aged women. Its most frequent form of presentation is asymptomatic with biochemical cholestasis and the presence of antimitochondrial antibodies (AMA). AIM: To describe the epidemiological characteristics, clinical presentation and treatment for patients with PBC at a clinical hospital. MATERIAL AND METHODS: Descriptive, observational, retrospective study, carried out between January 2015 and December 2020. Results: 179 patients (158 women) were cared in the study period. At the time of diagnosis, the median age was 54 years (range 24-76), 55% of them were asymptomatic, 45% had fatigue and 28% had pruritus. Positive AMA were present in 65% of patients, antinuclear antibodies (ANA) in 51%, and anti-smooth muscle antibodies (ASMA) in 9%. Immunoglobulin M (IgM) was elevated in 30% of the patients and 50% of patients were biopsied. Splenomegaly and esophageal varices were present in 24 and 22% of patients, respectively. PBC was associated with Sjogren's syndrome in 15%, hypothyroidism in 14%, osteoporosis in 13%, and scleroderma in 8%. CONCLUSIONS: The epidemiological characteristics of our patients agree with those published abroad. Laboratory cholestasis associated with the presence of AMA, currently allows diagnosis without the need for histological study. Ursodeoxycholic acid (UDCA) is the first-line treatment for patients with PBC. The use of biochemical response criteria is essential to identify patients who require other UDCA alternatives for isolated or combined treatment.

Estudo de prevalência de auto-anticorpos não órgão específicos em população rural do nordeste brasileiro (Cavunge-Ba) / Study of the prevalence of autoantibodies not specific national rural population of Northeast Brazil (Cavunge-Ba)

Os auto-anticorpos hepáticos (AAH), incluindo os anticorpos antinúcleo (ANA), antimúsculo liso (SMA), antimicrossoma de fígado e rim do tipo 1 (anti-M1), anticitosol hepático do tipo 1 (anti-LC1) e antimitocôndria (AMA), podem ser encontrados em 1 por cento a 43 por cento dos indivíduos saudáveis, sendo considerados auto-anticorpos naturais. Por outro lado, são também considerados marcadores de enfermidades auto-imunes, particularmente hepatite auto-imune (HAI) e cirrose biliar primária (CBP), duas doenças auto-imunes do fígado de prevalência ainda desconhecida na população brasileira. Com o intuito de avaliar a freqüência de AAH na população normal e sua correlação com estado de doença e de investigar a prevalência de HAI e CBP no Brasil, foram coletadas amostras de soro de 725 indivíduos, aleatoriamente selecionados, residentes no povoado de Cavunge para realização de AAH por imunofluorescência indireta (IFI), empregando cortes de roedores, fibroblastos e células Hep-2. Os resultados foram comparados com dados demográficos e marcadores sorológicos de infecção por vírus A, B e C. Positividade para AAH foi considerada na presença de titulação igualou superior a 1:40, sendo considerada clinicamente relevante titulação igualou superior a 1:80. Cento e um indivíduos (14 por cento) apresentaram reatividade para AAH, a maioria com títulos de 1:40, sendo observada positividade para SMA, ANA e AMA em respectivamente 10 por cento, 4 por cento e 0,1 por cento dos indivíduos. Não foi encontrada reatividade para anti-LKM 1 e anti-LC 1. Os principais padrões de IFI para SMA e ANA foram, respectivamente, vaso e glomérulo I e nuclear pontilhado grosso. Apenas um indivíduo com SMA exibiu reatividade para anti-microfilamento em baixo título, não sendo observado...

[Overlap syndrome autoimmune hepatitis=primary biliary cirrhosis. Comparative study with isolated autoimmune hepatitis results of a multicentric Tunisian]

Autoimmune hepatitis [AIR] and primary biliary cirrhosis [PBC] are two autoimmune diseases affecting the liver. Suggestive signs of the two diseases can however occur simultaneously or consecutively in the same patient leading to an overlap syndrome. The aim of our multicentric retrospective study was to study clinical, biological, immunological and histological features of 17 patients with overlap syndrome and to compare them with those of 62 patients with isolated autoimmune hepatitis. Overlap syndrome "AIH-PBC" accounts for 20,5%of patients with autoimmune hepatitis in our series. Our two groups were similar in age and gender. Clinically, in overlap syndrome group, a significantly higher prevalence of pruritus arid significantly lower prevalence of ascites were observed as compared to isolated AIH group. No significant differences were observed between the two groups of patients with regard to frequency of concurrent immune diseases. Biologically, serum alkaline phosphatase and garnma-glutamyl-transpeptidase were significantly higher in overlap syndrome than in patients with isolated AIH. Significantly more overlap syndrome patients were positive for anti-mitochondrial antibodies than isolated AIR patients. The patients with isolated AIR had a higher frequency of cirrhosis at presentation that the patients with overlap syndrome. The histologic profile was not different between isolated AIR and Overlap syndrome. Overlap syndrome "AIR-PBC" is not rare. Compared with isolated AIH, it is characterised by a higher frequency of clinical and biological signs of cholestasis, a higher frequency of antimitochondrial antibodies and the rarity of cirrhosis at the diagnosis of the disease

Cirrosis biliar primaria / Primary biliary cirrhosis

La cirrosis Biliar Primaria, es una enfermedad crónica, colestásica del higado, caracterizada por la destrucción inmunoinflamatoria de ductos biliares intrahepáticos. Es de curso progresivo y evoluciona a cirrosis, produciendo muerte por insuficiencia hepática o por hemorragia secundaria a ruptura de várices esófago-gástricas.