Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

Koyamada, Ryosuke; Higuchi, Takakazu; Kitada, Ayako; Nakagawa, Tomoko; Ikeya, Takashi; Okada, Sadamu; Fujita, Yoshiyuki

doi:10.2169/internalmedicine.54.4405

Cited by 9 publications

(7 citation statements)

References 19 publications

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“…29 Concomitant autoimmune liver diseases, including autoimmune hepatitis and primary biliary cirrhosis, as causes of cholestasis have only been reported in a few case reports describing hyperthyroidism in patients. 7,8,39,40 This indicates that concomitant autoimmune liver diseases are not a common cause of cholestasis in these patients. However, other underlying liver diseases should also be excluded in patients with hyperthyroidism and cholestasis, especially in high epidemic countries of hepatitis B or C virus.…”

Section: Discussionmentioning

confidence: 90%

“… 1 , 2 Severe cholestasis—either induced by thyrotoxicosis or as an adverse effect of ATD—has been increasingly reported in patients with hyperthyroidism. 3 – 6 Additionally, patients with Graves’ disease may also have concomitant complications in the form of other autoimmune diseases, such as autoimmune hepatitis 7 and primary biliary cirrhosis, 8 which can also present as cholestasis. Therefore, identification of the causes of cholestasis is important for appropriate management of patients with hyperthyroidism.…”

Section: Introductionmentioning

confidence: 99%

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Challenges in early identification of causes and treatment of cholestasis in patients with hyperthyroidism: a case report and literature review

et al. 2019

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Early identification of the causes of cholestasis is important for appropriate management of patients with hyperthyroidism. We report a patient who had hyperthyroidism and severe cholestasis after methimazole (MMI) treatment. The patient was diagnosed as having MMI-induced cholestatic hepatitis. Treatment with MMI was stopped at admission to hospital. However, his serum total bilirubin (TBil) level rose from 410.5 µmol/L to 519.9 µmol/L and prothrombin time activity (PTA) dropped from 81.0% to 52.2% in 10 days. To prevent further deterioration of his liver function, plasma exchange was performed three times, and dexamethasone (10 mg, intravenously) was used each time. His PTA rose to 101% and his TBil continued to increase to 669.8 µmol/L after plasma exchange. He was subsequently diagnosed as having thyrotoxicosis-induced cholestasis and treated with radioactive iodine (380 MBq) 2 weeks after admission. His hyperthyroidism was significantly relieved, but the TBil level further increased to 776.8 µmol/L. Three weeks after admission, oral prednisone (30 mg/day) was used in this patient. Subsequently, his TBil levels gradually decreased and his liver function almost normalized within 3 months. We discuss the literature on cholestasis in the context of hyperthyroidism.

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Section: Discussionmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Challenges in early identification of causes and treatment of cholestasis in patients with hyperthyroidism: a case report and literature review

et al. 2019

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“…Therefore, extrahepatic autoimmunity represents a continuous spectrum of autoimmunity involving liver and extrahepatic tissues. [9][10][11] We could not identify any reports of patients with autoimmune liver diseases who were diagnosed with PNH. PNH is caused by an acquired defect of the erythrocyte membrane that results in complement-mediated hemolysis.…”

Section: Discussionmentioning

confidence: 99%

Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome in a patient with paroxysmal nocturnal hemoglobinuria: a case report

et al. 2021

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Primary biliary cirrhosis (PBC)–autoimmune hepatitis (AIH) overlap syndrome is frequently associated with extrahepatic autoimmune disorders. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disease that is characterized by complement-mediated hemolysis due to erythrocyte membrane defects. However, autoimmune liver disease was not previously reported to be associated with PNH. A 37-year-old female patient was referred to our hospital with elevated liver enzymes and hematuria. On the basis of the symptoms and results of laboratory tests, radiographic studies, and pathologic results, she was diagnosed with PBC–AIH overlap syndrome and PNH. She was treated with a combination of ursodeoxycholic acid and prednisolone. The patient was symptom-free, with laboratory findings within near-normal range. The patient had recovered well at the 24-month follow-up evaluation. While we acknowledge that this was a single case, these findings expand our knowledge of immunological diseases that are associated with PNH and suggest an immune-mediated pathogenic pathway between PNH and PBC–AIH overlap syndrome. The combination of ursodeoxycholic acid and prednisolone can achieve therapeutic success. Routine follow-up of these patients is necessary to document disease progression.

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“…The prevalence of ITP among patients with AIH in Japan is reported to be 0.6% [2], and there are numerous previous case reports of AIH associated with ITP [5–12]. Although the frequency of autoantibodies against platelet surface GP in patients with AIH remains poorly understood [7–9], some previous reports described that HLA class II genes, which are associated with susceptibility to AIH, influence the production of anti-GP antibody specificities, suggesting a relationship between AIH and ITP [13, 14].…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Hepatitis with Concomitant Idiopathic Thrombocytopenic Purpura Diagnosed by Transjugular Liver Biopsy

Fukuda

Takata

Kitaguchi

et al. 2018

Case Reports in Hepatology

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Patients with autoimmune hepatitis (AIH) may sometimes have concomitant idiopathic thrombocytopenic purpura (ITP). Severe thrombocytopenia in ITP interferes with percutaneous liver biopsy for pathological diagnosis of AIH. Here, we report a case of AIH with ITP in a 63-year-old woman. The patient presented to our hospital with liver dysfunction and thrombocytopenia. For histological examination, transjugular liver biopsy (TJLB) was performed, leading to a diagnosis of AIH. Corticosteroids treatment led to an improvement in her liver enzyme levels and platelet count. In conclusion, patients with AIH may sometimes have concomitant ITP. TJLB was effective for making the diagnosis of AIH with severe thrombocytopenia due to ITP.

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Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

Cited by 9 publications

References 19 publications

Challenges in early identification of causes and treatment of cholestasis in patients with hyperthyroidism: a case report and literature review

Challenges in early identification of causes and treatment of cholestasis in patients with hyperthyroidism: a case report and literature review

Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome in a patient with paroxysmal nocturnal hemoglobinuria: a case report

Autoimmune Hepatitis with Concomitant Idiopathic Thrombocytopenic Purpura Diagnosed by Transjugular Liver Biopsy

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