Acute Liver Failure Because of Wilson Disease With Overlapping Autoimmune Hepatitis Features

Loudianos, Georgios; Zappu, Antonietta; Lepori, Maria Barbara; Dessì, Valentina; Mameli, Eva; Orrù, Sandra; Podda, Rosa Anna; Virgiliis, Stefano De

doi:10.1097/mpg.0000000000000557

Cited by 11 publications

(18 citation statements)

References 8 publications

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“…Acute hepatitis caused by WD with AIH markers was also reported, with generally bad outcomes associated with diagnosis delay. The role of autoantibodies in WD is still unknown; it could be an early feature of its pathological mechanism, due to hepatocyte necrosis or even a simultaneous presentation of both entities 10 11. For this, evaluating AIH patients for WD could be life saving when facing inadequate response to initial therapy 12…”

Section: Discussionmentioning

confidence: 99%

Wilson’s disease presentation resembling autoimmune hepatitis

et al. 2019

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We report a case of a 25-year-old female patient who showed chronic hepatopathy with elevated levels of autoantibodies and gamma globulins, resembling autoimmune hepatitis. After 8 weeks of unsuccessful immunosuppressive treatment, further evaluation showed laboratorial and histological findings suggestive of Wilson’s disease. The new treatment with D-penicillamine resulted in positive outcome, despite the initial misleading diagnosis.

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Section: Discussionmentioning

confidence: 99%

Wilson’s disease presentation resembling autoimmune hepatitis

et al. 2019

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“…A similar case was presented by Loudianos et al -a 15-year-old girl with ALF and confirmed WD (11 FS points) also revealed some autoimmune features. Despite including treatment for both WD and AIH, her condition got worse and she underwent successful liver transplantation [22]. Those two cases show that ALF due to WD can present with AIH features, so patients presenting with ALF and positive auto-antibodies require a rapid diagnostic approach for WD.…”

Section: Discussionmentioning

confidence: 96%

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

Naorniakowska¹,

Woźniak²,

Pronicki³

et al. 2020

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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.

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“…Out of 10 studies, there were 8 case reports [7,9,10,18,[20][21][22][23], 1 opinion study [24], and 1 letter to the editor [25]. So that 6 case report studies, 1 opinion study, and the letter to the editor reported one case [9, 10,18,20,[22][23][24]. The other two studies reported two [21], and four cases [7], respectively.…”

Section: Study Characteristicsmentioning

confidence: 99%

“…Diagnosis direction was autoimmune hepatitis to Wilson disease in 8 cases [7,9,21,23,24], and reverse in 3 cases [7,20,22]. In three cases [10,18,25], the simultaneity of Wilson disease and autoimmune hepatitis was considered from the beginning with no primary and secondary diagnosis. Finally, out of 8 patients [7,15,21,23,24] diagnosed with autoimmune hepatitis to Wilson disease, in two cases [9, 23], the primary diagnosis of autoimmune hepatitis was rejected.…”

Section: Study Characteristicsmentioning

confidence: 99%

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Simultaneous Presentation of Autoimmune Hepatitis and Wilson's Disease: A Systematic Review Study

et al. 2021

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Background: The specialists should identify the features of Wilson disease and autoimmune hepatitis when both affect a patient to adopt appropriate treatment. Objectives: This study was conducted to determine features of the patient, disease, diagnostic studies, and therapeutic measures in cases of simultaneity of Wilson disease and autoimmune hepatitis. Methods: To find evidence related to the study objectives, we searched databases such as Barakat knowledge network system, SID, Magiran, Google Scholar, Web of Science, ProQuest, Springer, ScienceDirect, Medline via PubMed, and Scopus with specified Persian and English keywords, including “Wilson’s Disease”, “Autoimmune”, and “Hepatitis”. The inclusion criteria for the studies were 1) the study was observational and 2) the study was published in Persian or English. The exclusion criteria included low-quality studies based on the score obtained from the checklist. The obtained studies were screened in terms of titles, abstracts, and full text, and finally, the qualified studies entered the review process. The relevant data were extracted according to a designed checklist. Results: Finally, 10 studies were included in the review process. Information about 14 patients was reported. The Mean±SD age of the participants in the studies was 19±11 years. The direction of diagnosis was from autoimmune hepatitis to Wilson disease in 8 cases and from Wilson disease to autoimmune hepatitis in 3 cases. The simultaneity of autoimmune hepatitis and Wilson disease was considered in 3 patients with no primary and secondary diagnosis. Conclusions: The comorbidity of Wilson disease and autoimmune hepatitis is uncommon but is important. In the presence of relevant symptoms in these patients, the comorbidity of these two diseases should be considered. Accordingly, additional assessments such as serum ceruloplasmin, urinary 24-h copper, molecular genetic testing, MRI, serological tests, anti-nuclear antibody, anti-mitochondrial antibody, anti-smooth muscle antibody, complement level, gamma globulin, IgG, albumin, Kayser-Fleischer ring eye examination, and liver biopsy should be considered for correct diagnosis. If appropriate treatment was started for the disease with a diagnosis of Wilson disease or autoimmune hepatitis, but the response to treatment was insufficient, it is better to consider the simultaneous occurrence of two diseases or the initial misdiagnosis.

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Acute Liver Failure Because of Wilson Disease With Overlapping Autoimmune Hepatitis Features

Cited by 11 publications

References 8 publications

Wilson’s disease presentation resembling autoimmune hepatitis

Wilson’s disease presentation resembling autoimmune hepatitis

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

Simultaneous Presentation of Autoimmune Hepatitis and Wilson's Disease: A Systematic Review Study

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