Postinfantile Giant Cell Hepatitis with Features of Acute Severe Autoimmune Hepatitis Probably Triggered by Diclofenac in a Patient with Primary Myelofibrosis

Arvaniti, Pinelopi; Zachou, Kalliopi; Koukoulis, George Κ.; Dalekos, Georgios N.

doi:10.1155/2018/9793868

Cited by 7 publications

(12 citation statements)

References 46 publications

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“…However, the link between paramyxovirus and steroids also appear to be effective in inducing remission of PGCH as well as the underlying disease (1). Similarly, patients with drug-induced PGCH make a full recovery once the offending drug is withdrawn (1,9,10,18). Patients with idiopathic PGCH generally have a poor prognosis because there is no targeted therapy for PGCH.…”

Section: Discussionmentioning

confidence: 99%

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Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature

Xiao

Gao

Deschênes

2021

CanLivJ

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Giant cell hepatitis (GCH) is a rare entity in adults that is characterized by large multinucleated hepatocyte formation and parenchymal inflammation. We present a case of acute liver failure in a 33-year-old woman secondary to autoimmune hepatitis (AIH). A liver biopsy revealed submassive hepatocyte necrosis consistent with GCH. We conducted a literature review of 187 reported cases of post-infantile GCH in adults. AIH was the most commonly reported cause of GCH, but GCH was associated with a wide spectrum of etiologies, including infections, rheumatological diseases, hematological diseases, malignancies, and medications. The severity of disease can range from mild hepatitis to fulminant hepatic failure. The mortality rate among the cases in the literature was 18.82%. GCH is managed by treating the underlying cause, and ribavirin has been proposed as a treatment option for idiopathic GCH. A small number of patients progress to requiring orthotopic liver transplant, but recurrence is possible post-transplant.

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Section: Discussionmentioning

confidence: 99%

“…Patients have also developed PGCH after exposure to a variety of drug or herbal products (1,14,18). Some drugs may lead to PGCH by triggering AIH (9). For other drugs, however, the pathophysiology leading to PGCH is still not known.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature

Xiao

Gao

Deschênes

2021

CanLivJ

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“…20 Some cases of PIGCH had features of autoimmune disorders (e.g., elevated autoantibodies) and improved after immunosuppressive therapy without having a specific autoimmune disease diagnosed. 11,36 Viruses associated with PIGCH included hepatitis C (HCV), 15,16,29,[37][38][39] human immunodeficiency virus (HIV), 16,28,39 human herpes virus-6 (HHV-6), 40 cytomegalovirus (CMV), 14,41,42 Epstein-Barr virus (EBV), 43,44 herpes simplex virus (HSV), 29 paramyxoviruses, 8,33,43,45 hepatitis A, 38,46,47 hepatitis B, 38 and hepatitis E. 48,49 In addition to medications, such as clometacin, 50 diclofenac, 51 doxycycline, 11,17 amoxicillin/clavulanate, 12 dehydrocholic acid, 11 and testosterone analogue, 52 herbal remedies, [53][54][55] and dietary supplements 56 were also reported to have an association with PIGCH. The most commonly associated malignancy with PIGCH appeared to be chronic lymphocyte leukemia (CLL).…”

Section: Distribution Of the Etiologymentioning

confidence: 99%

“…The most commonly associated malignancy with PIGCH appeared to be chronic lymphocyte leukemia (CLL). 37,43,44,51,[57][58][59][60][61][62] In addition, other malignancies seen in patients with PIGCH included Hodgkin's lymphoma, papillary thyroid carcinoma, 63 anaplastic carcinoma, 58 and primary myelofibrosis. 51 Some patients had more than one etiology, including autoimmune disorder and a viral infection, 28,29,33 autoimmune disorder and medication, 11 CLL with autoimmune hemolytic anemia, and CLL with a viral infection.…”

Section: Distribution Of the Etiologymentioning

confidence: 99%

Post-infantile Giant Cell Hepatitis: A Literature Review and Meta-analysis

Jiao¹,

Zhang²

2022

J Clin Transl Pathol

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Post-infantile giant cell hepatitis (PIGCH) is a rare disease entity in adults with a multifactorial etiology and widely variable clinical courses and outcomes. The factors associated with the worse outcomes of this disease entity are still unclear. We identified 68 PIGCH patients by searching PubMed and performed meta-analysis. Among the 68 patients, 32% of the cases were associated with autoimmune disorders, followed by 21% associated with viral infections, 10% with medication, and 7% with malignancy. Twenty-four percent of the patients had more than one etiological factor, and 6% had other uncommon etiologies or an etiology that could not be identified. At the time of this report, 17 patients had died of the disease (poor outcome), and 51 patients remained alive with the disease (good outcome). Compared to the patients with a good outcome, the patients with a poor outcome were characterized by older age, lower levels of platelets and albumin, higher level of total bilirubin, and a diffuse distribution pattern of giant cells in the liver. There were no differences in gender distribution, aminotransferase, alkaline phosphatase, gamma-glutamyl transferase, etiological distribution, or other histological features, including interface hepatitis, necrosis, lobular inflammation, portal inflammation, cholestasis, or fibrosis. Further studies would be needed to better understand the disease mechanisms and unmask any additional etiological factors and targeted therapies.

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“…AIH may also develop after the administration of many drugs, supplements and/or herbals, with nitrofurantoin and minocycline being the best documented among diverse cases (Table 4) [40-44]. Drug-induced AIH is a complex and challenging condition characterized clinically and histologically by different phenotypes across the disease spectrum [40,45].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Hellenic Association for the Study of the Liver Clinical Practice Guidelines: Autoimmune hepatitis

Dalekos¹,

Koskinas

Papatheodoridis

2018

aog

152

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Autoimmune hepatitis (AIH) is a relatively rare acute or chronic liver disease of unknown etiology characterized by large heterogeneity. Its distribution is global, covering all ages, both sexes and all ethnic groups. The aim of the present Clinical Practice Guidelines (CPG) of the Hellenic Association for the Study of the Liver was to provide updated guidance and help to gastroenterologists, hepatologists, internists and general practitioners for AIH diagnosis and management. AIH diagnosis is based on clinicopathological characteristics: namely, polyclonal hypergammaglobulinemia, particularly of immunoglobulin G (IgG), circulating autoantibodies, interface hepatitis on liver histology, absence of viral hepatitis, and a favorable response to immunosuppression. Clinical manifestations at disease onset are variable, ranging from asymptomatic to the acute/severe form. Aminotransferase and bilirubin levels vary, while the presence of hepatitis at the histological level is a prerequisite for diagnosis. Autoantibodies are the hallmark for AIH diagnosis; therefore, the CPG describe the appropriate serological algorithm for their detection. AIH therapy should aim to achieve complete biochemical (normalization of IgG and aminotransferases) and histological remission. All patients who have active disease, even those with cirrhosis, should be treated with individualized and response-guided induction therapy using prednisolone in combination with azathioprine or mycophenolate mofetil as first-line therapy. Immunosuppression should be given for at least 3 years and for at least 2 years after the achievement of complete biochemical response, while a liver biopsy should be recommended before treatment discontinuation. Current CPG are also provided for several specific conditions and difficult-to-treat patients.

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Postinfantile Giant Cell Hepatitis with Features of Acute Severe Autoimmune Hepatitis Probably Triggered by Diclofenac in a Patient with Primary Myelofibrosis

Cited by 7 publications

References 46 publications

Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature

Autoimmune post-infantile giant cell hepatitis: a case report and review of the literature

Post-infantile Giant Cell Hepatitis: A Literature Review and Meta-analysis

Hellenic Association for the Study of the Liver Clinical Practice Guidelines: Autoimmune hepatitis

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