A retrospective analysis of patients with autoimmune hepatic diseases: a study from Charlotte Maxeke Johannesburg academic hospital ICMJAH)
Date
2018
Authors
Ostrofsky, Marc Ilan
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Abstract
Background: Overlap syndromes (OS) of the autoimmune liver diseases (AILD's) are characterized by the coexistence of features of autoimmune hepatitis (AIH) and features of cholestatic liver disease i.e. primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). There is a paucity of information with regards to characterizing these OS in the South African context.
Aim: Compare the clinical features and outcomes of autoimmune hepatitis primary biliary cirrhosis overlap syndrome (AIH/PBC OS), and autoimmune hepatitis primary sclerosing cholangitis overlap syndrome (AIH/PSC OS) with each other and also to the individual disease entities i.e. AIH, PBC and PSC.
Methods: This was a retrospective record review of patients at the liver clinic at CMJAH with the diagnosis of AIH, PBC, PSC, AIH/PBC OS and AIH/PSC OS during the period 01/01/1990 - 31/12/2016. The demographics, clinical characteristics, biochemical, histological and radiological results as well as treatment and outcomes were collected. A data collection tool was used. The overlap syndromes were compared with the individual diseases and with each other. Results: There were 97 patients eligible for inclusion in the study. Forty four patients were diagnosed with AIH; 29 with PSC; four with PBC; 10 with AIH/PSC OS and 10 with AIH/PBC OS.
The majority of the cohort were Black i.e. 53 patients; 29 patients were White, 13 patients were Indian and two patients were Coloured. In the AIH/PSC OS group there were a higher proportion of Black patients (6/10). The racial profile of patients in the AIH/PBC OS group was different in that the majority were White (7/10). The AIH/PSC OS group had the best outcome (50% remission). This was followed by the AIH group (32% remission) and the PSC group where the remission rate achieved was 14%. Importantly, there were no cases of remission in the AIH/PBC OS group.
Conclusions: Based on the results of the current study it can be surmised that ethnicity could influence the development of specific overlap syndromes [AIH/PBC OS (70% White) and AIH/PSC OS (60% Black)]. The liver enzyme profile alone cannot be used to differentiate between the OS. In our population AIH/PSC OS and AIH have better clinical outcomes and a lower complication rate compared both to AIH/PBC and PSC and Autoimmune hepatitis/ primary biliary cirrhosis overlap syndrome has a poorer prognosis than either AIH or PBC alone. Early recognition of the OS may assist in timely use of combination drugs which may improve outcomes It was difficult to draw any meaningful conclusions from the comparison between PBC and the other diseases as there were only four patients with PBC.
Based on the literature together with the results of this retrospective study, it is important that definitive diagnostic criteria are developed in order to differentiate the autoimmune liver disease overlap syndromes from the separate disease entities. Further large volume prospective trials are warranted to better differentiate between the overlap syndromes and their single disease entities.
Description
A research report submitted to the Faculty of Health Sciences, University of Witwatersrand, Johannesburg, in partial fulfillment of the requirements for the degree of Master of Medicine in the branch of Internal Medicine Johannesburg 2018