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

Marlen Ivón Castellanos Fernández; Maria Edelina Cepeda Mullo; Deyanira la Rosa Hernández; Hector Vega Sánchez; Licet González Fabian; Robert G Gish; Ana Luisa Torres González; Zaily Dorta Guridi

doi:10.1016/j.curtheres.2020.100594

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

Curr Ther Res Clin Exp. 2020 Aug 13:93:100594. doi: 10.1016/j.curtheres.2020.100594. eCollection 2020.

Authors

Marlen Ivón Castellanos Fernández¹, Maria Edelina Cepeda Mullo¹, Deyanira la Rosa Hernández², Hector Vega Sánchez², Licet González Fabian³, Robert G Gish⁴, Ana Luisa Torres González⁵, Zaily Dorta Guridi⁵

Affiliations

¹ Department of Research and Teaching, National Institute of Gastroenterology, Havana, Cuba.
² Department of Immunology, National Institute of Gastroenterology, Havana, Cuba.
³ Department of Pathology, National Institute of Gastroenterology, Havana, Cuba.
⁴ Las Vegas, Nevada Hepatitis B Foundation, University of Nevada, Las Vegas, Las Vegas, Nevada.
⁵ Department of Hepatology, National Institute of Gastroenterology, Havana, Cuba.

Abstract

Background: Population-based studies on the clinical course and prognosis of autoimmune hepatitis (AIH) from Caribbean countries are limited.

Objective: The aim of this study was to provide information regarding the clinical and laboratory findings, histological profile, treatments, and outcomes of patients with AIH with long-term follow-up in a tertiary referral center.

Methods: A retrospective study was performed at the National Institute of Gastroenterology in Havana, Cuba, by enrolling 82 patients with a well-documented, long-term clinical course of AIH. Clinical and laboratory findings, histological profiles, treatments, and outcomes were analyzed.

Results: At diagnosis, 73 (89%) patients had AIH type 1, 84.1% were women, and their median age was 46.5 years (range, 17-79 years). The median follow-up period was 84 months (interquartile range, 12-276 months). Clinical onset was mild or subclinical in 72% of patients and asymptomatic in 12.2%. At diagnosis, the Hennes's median score was 6 (range, 3-8). Complications were seen in 44 (53.6%) patients, 42 (51.2%) with liver-related complications and 9 (10.9%) without liver-related complications. Cirrhosis was present at diagnosis in 32 (39%) patients. Cirrhosis was subsequently diagnosed in the other 28 patients who were not cirrhotic at diagnosis, over a median follow-up of 12 (IQR, 2-84) months. During follow-up, 6 patients died (7.3%). Cumulative survival at 5 and 10 years was 98.4% and 89%, respectively. A complete biochemical response was achieved in 79% of patients in a mean (SD) of 11.7 (11.6) months. Side effects due to treatment were reported in 76 (92.7%) patients, and no pretreatment factors were found to predict treatment response.

Conclusions: These Cuban patients with AIH had acceptable disease remission rate and a prompt treatment response. Although most patients had advanced-stage liver disease at diagnosis or developed during follow-up, the cumulative survival rate was high when patients were receiving and complying with treatment.

Keywords: Autoimmune hepatitis; Azathioprine; Complications; Follow up; Prednisone; Survival.