Wilson's disease with coexisting autoimmune hepatitis

Yener, Serkan; Akarsu, Mesut; Karacanci, Cuneyt; Şengül, Bülent; Topalak, Ömer; Biberoğlu, Kutay; Akpınar, Hale

doi:10.1111/j.1440-1746.2004.03254.x

Cited by 31 publications

(27 citation statements)

References 2 publications

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“…Occasional patients with WD may benefit from a brief course of treatment with corticosteroids along with appropriate specific treatment for WD 26. In some patients concurrent WD and autoimmune hepatitis cannot be excluded 133. Hepatic steatosis in WD is rarely as severe as in nonalcoholic fatty liver disease (NAFLD).…”

Section: Clinical Featuresmentioning

confidence: 99%

Diagnosis and treatment of Wilson disease: An update

Roberts¹,

2008

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Section: Clinical Featuresmentioning

confidence: 99%

Diagnosis and treatment of Wilson disease: An update

Roberts¹,

2008

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“…However, several case reports have described AIH with superimposed or coexisting Wilson disease [54] and up to 17.2% of patients diagnosed with AIH may have a heterozygous C282Y mutation [55] . Presence of hemosiderin granules and evaluation of their cellular and subcellular distribution patterns are very helpful in assessing concurrent siderosis in AIH patients.…”

Section: Concurrence With Other Metabolic Diseasesmentioning

confidence: 99%

Role of Histopathology in Autoimmune Hepatitis

Tiniakos

Brain

Bury

2015

Dig Dis

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The diagnosis of autoimmune hepatitis (AIH) is based on a combination of biochemical, immunological and histological features and exclusion of other causes of liver disease. Typical histological features include a chronic hepatitis pattern of injury with portal inflammation and interface activity, predominance of plasma cells in the portal infiltrate, emperipolesis, and hepatocellular rosette formation. Centrilobular injury with prominent hepatocellular necrosis and mononuclear inflammation is now recognised in the histological spectrum of AIH and may represent an early stage of the disease. Liver histology plays a major role in clinical diagnostic scoring systems and is important to confirm or support the clinical diagnosis of AIH. This review focuses on the role of histopathology in AIH and highlights the contribution of histological interpretation to the diagnosis of AIH, differential diagnosis from other entities, recognition of concurrent liver disease, and identification of the so-called overlap or variant syndromes, and addresses the importance of liver biopsy in the management and prognosis of patients with AIH.

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“…1 Conditions having features of two different autoimmune liver diseases are grouped as 'Overlap Syndromes'. 3 But there is no current agreement on what are the specific diagnostic criteria for an overlap. 4 Type-I AIH has anti-nuclear antibodies (ANA) and/or antismooth muscle antibodies (ASMA), whilst type-II AIH is has anti-liver kidney microsomal type-1 (anti-LKM-1) antibodies.…”

Section: Discussionmentioning

confidence: 99%

“…1 The coexistence of Wilson's disease (WD) and Autoimmune Liver Disease (ALD) in same patients is a rare entity. 2,3 Hepatocyte necrosis and intracellular antigen exposure to immune system is seen in WD and results in low titer autoantibody production. 4 Therefore, it is highly recommended to screen WD in patients labelled as AIH, especially when the response to treatment with immunosuppressant drugs is disappointing.…”

Section: Introductionmentioning

confidence: 99%

Wilsons disease and autoimmune liver disease overlap syndrome: a clinical study

et al. 2019

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Background: The coexistence of Wilson’s disease and autoimmune liver disease in a same patient is a rare entity. Combined treatment with steroid and D-penicillamine may be effective. Aim of the study was analyse the clinical, histological, laboratory profile for patients with chronic liver disease with aim of finding the etiology of the disease.Methods: It is an observational study. Common clinical presentations were evaluated. Laboratory investigations done include complete blood count, renal and liver function tests, prothrombin time, viral markers for hepatitis A, B, C and E, USG abdomen and pelvis, portal Doppler studies and upper GI endoscopy. Specific tests include ANA, AMA, ASMA, Anti LKM-1Ab, serum ceruloplasmin and 24hrs urinary copper were done. Liver biopsy was done in selected patients.Results: Commonest clinical presentation was abdominal distension (80%), abdominal pain (30%), pedal edema (60%), splenomegaly (40%) and upper GI bleed (40%). Laboratory investigation revealed anemia (50%), thrombocytopenia (70%), prothrombin time prolongation in (60%), normal liver function in 60%, abnormal liver function in (40%). Autoimmune markers revealed ANA strong positivity in (40%), mild positivity in (60%). AMA, ASMA, Anti-LKM-1 were negative in all cases (100%). Liver biopsy showed features of autoimmune liver disease and Periportal copper deposition in 80% of cases.Conclusions: Coexistence of Wilson’s disease and autoimmune liver disease is a rare entity and medical treatment with steroids and D-penicillamine simultaneously to be started in these patients.

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Wilson's disease with coexisting autoimmune hepatitis

Cited by 31 publications

References 2 publications

Diagnosis and treatment of Wilson disease: An update

Diagnosis and treatment of Wilson disease: An update

Role of Histopathology in Autoimmune Hepatitis

Wilsons disease and autoimmune liver disease overlap syndrome: a clinical study

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