The Value of Autoimmune Hepatitis Scoring Systems in Differentiationbetween Autoimmune Hepatitis and Wilson Disease
Nermine Mohammed Mohammed Elsayed;
Abstract
Autoimmune hepatitis (AIH) is an inflammatory condition of the liver that can affect patients of all ages, sexes, and races (Boberg et al., 1998). It is characterized serologically by high levels of transaminases and immunoglobulin G (Ig), and the presence of autoantibodies; and characterized histologically by interface hepatitis, in the absence of a known etiology (Vergani et al., 2007).
Early diagnosis may be difficult because the clinical picture is heterogeneous and there is no specific test applicable for all patients(Feld et al., 2005).
Wilson disease is a metabolic disorder characterized by excessive copper deposition, predominantly in the liver and brain.
WD in children may be obscure and requires extensive investigations to establish the diagnosis (Manolaki et al., 2009).
WD may present with a hepatic disorder that resembles AIH.
If the clinical features of WD resemble AIH; findings in liver biopsy may also suggest an autoimmune process, the classic piecemeal necrosis may be present (Sanyal, 2011).
In 1993, the International Autoimmune Hepatitis Group (IAIHG) proposed diagnostic criteria for AIH, which were revised in 1999 (Alvarez et al., 1999). These criteria were devised primarily by expert consensus and introduced to allow comparison of studies from different centers. Because these criteria are complex, insufficiently validated, and include a variety of parameters of questionable value, the IAIHG decided to devise a simplified scoring system for wider applicability in routine clinical practice based on the data of patients with well-established diagnosis (Hennes et al., 2008).
This retrospective study included data retrieved from the files of WD & AIH patients who were following up in pediatrics department hepatology clinic Ain Shams University and Yassin Abdel Ghaffar (YAG) charity centre for liver disease & research. Its aim was to assess the ability of original and simplified AIHSS to differentiate between patients with autoimmune hepatitis and those with Wilson disease.
Early diagnosis may be difficult because the clinical picture is heterogeneous and there is no specific test applicable for all patients(Feld et al., 2005).
Wilson disease is a metabolic disorder characterized by excessive copper deposition, predominantly in the liver and brain.
WD in children may be obscure and requires extensive investigations to establish the diagnosis (Manolaki et al., 2009).
WD may present with a hepatic disorder that resembles AIH.
If the clinical features of WD resemble AIH; findings in liver biopsy may also suggest an autoimmune process, the classic piecemeal necrosis may be present (Sanyal, 2011).
In 1993, the International Autoimmune Hepatitis Group (IAIHG) proposed diagnostic criteria for AIH, which were revised in 1999 (Alvarez et al., 1999). These criteria were devised primarily by expert consensus and introduced to allow comparison of studies from different centers. Because these criteria are complex, insufficiently validated, and include a variety of parameters of questionable value, the IAIHG decided to devise a simplified scoring system for wider applicability in routine clinical practice based on the data of patients with well-established diagnosis (Hennes et al., 2008).
This retrospective study included data retrieved from the files of WD & AIH patients who were following up in pediatrics department hepatology clinic Ain Shams University and Yassin Abdel Ghaffar (YAG) charity centre for liver disease & research. Its aim was to assess the ability of original and simplified AIHSS to differentiate between patients with autoimmune hepatitis and those with Wilson disease.
Other data
| Title | The Value of Autoimmune Hepatitis Scoring Systems in Differentiationbetween Autoimmune Hepatitis and Wilson Disease | Other Titles | قيمة نظام القياسالنقاطىلمرضالتهابالكبدذاتىالمناعة فى التفرقة بينمرضالتهابالكبدذاتىالمناعةومرضويلسون | Authors | Nermine Mohammed Mohammed Elsayed | Issue Date | 2014 |
Recommend this item
Similar Items from Core Recommender Database
Items in Ain Shams Scholar are protected by copyright, with all rights reserved, unless otherwise indicated.