Diagnosis and Treatment of Autoimmune Liver Diseases in a Tertiary Referral Center in Cuba

Abstract

BackgroundAutoimmune 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.ObjectivesThe 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.MethodsA 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.ResultsOf 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%).ConclusionsThe 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.

A 10-year-old girl presenting with jaundice, deterioration of school performance and itching

“…[15][16][17] In autoimmune hepatitis, progressive immune mediated inflammatory tissue damage occurs due to loss of self-tolerance. 18 Autoimmune hepatitis can occur at any age group, the peak incidence occurs at the ages of 16 to 30 years. [10][11][12][13]20 Females are affected more than males, ratio of 3.6:1.…”

Particularities of Autoimmune Hepatitis in Latin America

“…Despite this subclinical presentation in Caucasian patients, one‐third of all adult patients and almost 50% of children already have cirrhosis at the time of diagnosis, and these frequencies are lower than those found in the LA series, whose incidence of cirrhosis at diagnosis varies from 35.5% to 62.3%, according to the criteria used for its diagnosis (clinical/laboratory/radiological versus histological). In Table , some data about the clinical presentation of AIH in some countries of LA are depicted.…”

“…The pathogenesis of the disease is not completely understood and involves a complex interaction between genetic predisposition and environmental triggers with cross‐reactivity with structurally similar autoantigens, leading to loss of immunotolerance. AIH is responsible for 56.6% of autoimmune liver diseases in Cuba, 4% to 6% of adult liver transplants in Peru, and is the second most common causative factor of acute liver failure in Argentina (25% of the cases) …”

Autoimmune Hepatitis in Cuban Patients: A Retrospective Analysis of Clinical and Histological Profiles, Treatments, and Outcomes

“…A prospective Cuban study among 106 patients with autoimmune liver diseases identified AIH as the most common condition in 56 patients, 6% followed by primary biliary cholangitis in 20 patients, 7%, but it does not provide enough information about AIH and its differential characteristics, which range from the diagnosis to treatment and outcomes. 11 The authors of this study aimed to provide details about the clinical and laboratory findings, histological profile, treatments, and outcomes of patients with AIH in a tertiary referral center in a long-term follow-up study. The data will expand the knowledge of this disease in Cuba and Latin America.…”