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ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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1/2020
vol. 95
 
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abstract:
Original paper

Autoimmune hepatitis, Wilson’s disease, or both? An analysis of challenging cases

Magdalena Naorniakowska
1
,
Małgorzata Woźniak
2
,
Maciej Pronicki
2
,
Wiesława A. Grajkowska
2
,
Diana Kamińska
1
,
Wojciech Jańczyk
1
,
Maciej Dądalski
1
,
Bożena Cukrowska
2
,
Piotr Socha
1

  1. Department of Gastroenterology, Hepatology, Nutritional Disorders and Paediatrics, The Children’s Memorial Health Institute, Warsaw, Poland
  2. Department of Pathology, The Children’s Memorial Health Institute, Warsaw, Poland
Pediatr Pol 2020; 95 (1): 18–24
DOI: https://doi.org/10.5114/polp.2020.94913
Online publish date: 2020/03/31
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Introduction
The comorbidity of autoimmune hepatitis (AIH) and Wilson’s disease (WD) can be considered, but has never been proven based on therapeutic response. There is a risk of misdiagnosis with standard diagnostic approaches. We describe a case of a challenging patients where final diagnosis could not be established with primary diagnostic approaches, and we question the coexistence of AIH and WD.

Material and methods
We identified four cases among 165 patients with WD and 321 with AIH treated in our hospital, whose primary diagnosis was changed or questioned, analysis of which shows the difficulty in differentiating between these two diseases.

Results
We presented four patients – three of them were diagnosed because of elevated serum aminotransferases activity and one due to acute hepatic failure. The three children were initially diagnosed as AIH, but after meticulous screening, WD was diagnosed. In the fourth case, WD was diagnosed from the beginning, but some autoimmune features and an incomplete response to the treatment prompted us to use steroids to achieve full recovery.

Conclusions
WD can usually be distinguished from AIH, but in selected cases differential diagnosis is challenging. It seems to be extremely important to carry out a complete diagnostic set for WD in patients with initial diagnosis of AIH with poor response to steroids. In a large cohort of patients with WD and with AIH, only one seemed to have comorbidity, but even in this case AIH was not fully confirmed.

keywords:

hepatitis, comorbidity, differential diagnosis, autoantibodies