Giant-cell Hepatitis—Rare Entity in Adults

Shetty, Shiran; Janarthanan, Krishnaveni; Leelakrishnan, Venkatakrishnan; Nirmala, V

doi:10.1016/j.jceh.2016.02.007

Cited by 7 publications

(11 citation statements)

References 9 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 Similarly, patients with drug-induced PGCH make a full recovery once the offending drug is withdrawn (1,9,10,18).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%

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%

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

Xiao

Gao

Deschênes

2021

CanLivJ

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“…Auch infektiöse Erkrankungen können verantwortlich sein, z. B. durch Paramyxoma-, Hepatitis-(A, B, C, E) oder Herpesviren (HHV6, EBV), humane Papillomviren sowie das Humane Immundefizienz-Virus [1,2,5,6]. Verschiedenste Medikamente sind ebenfalls als Auslöser der PIGCH beschrieben.…”

Section: Diskussionunclassified

Erfolgreiche Therapie einer idiopathischen postinfantilen Riesenzellhepatitis mit einem Calcineurininhibitor

Hof

Flohr

Thimme

et al. 2021

Dtsch Med Wochenschr

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Zusammenfassung Zusammenfassung Die Riesenzellhepatitis ist eine seltene, ätiologisch heterogene Erkrankungsgruppe mit dem gemeinsamen histomorphologischen Merkmal multinukleärer Riesenzellen. Häufig assoziiert ist sie mit Autoimmun- und Viruserkrankungen sowie Medikamenteneinnahme, weshalb die Therapie vornehmlich in der Behandlung der Grunderkrankung bzw. einer Immunsuppression besteht. Anamnese Die Vorstellung des 76-jährigen Patienten erfolgte mit der extern histologisch gesicherten Diagnose einer postinfantilen Riesenzellhepatitis und frustranem Therapieversuch mit Budesonid zur weiteren Therapie. Untersuchung Anamnestisch, laborchemisch, serologisch und histopathologisch ließ sich keine der Hepatitis zugrunde liegende Ursache eruieren. Therapie und Verlauf Unter immunsuppressiver Therapie mit zunächst Glukokortikoiden in Monotherapie, später Cyclophosphamid sowie auch intermittierend einem Anti-CD20-Antikörper gelang keine Erkrankungskontrolle, weshalb die Therapie mit dem Calcineurininhibitor Tacrolimus in Kombination mit einer niedrigdosierten Glukokortikoid-Therapie begonnen wurde. Hierunter konnte die Erkrankung stabilisiert werden. Folgerung Unsere Beobachtung einer guten Hepatitis-Kontrolle unter Tacrolimus unterstreicht die Rolle von Calcineurininhibitoren in der Behandlung der postinfantilen Riesenzellhepatitis, wobei über deren Einsatz bisher sehr wenige Daten vorliegen.

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“…Serological workup for A1AT deficiency, Wilson's disease, hemochromatosis, and granulomatosis with polyangiitis were also negative suggesting a possible autoimmune or drug-induced hepatitis. Table 1 indicates the patient's Roussel Uclaf Causality Assessment Method [12] . Under "assessment" are the variables that go into calculating the score.…”

Section: Hospital Coursementioning

confidence: 99%

“…The exact mechanism by which drug-induced liver injury (DILI) leads to PIGCH remains to be understood, however, the formation of giant cells is thought to be an idiosyncratic reaction in response to tissue injury [12] . When DILI occurs, destruction of liver parenchyma leads to the exposure of liver proteins inducing an autoimmune phenomenon.…”

Section: Assessmentmentioning

confidence: 99%

“…In addition to the varied clinical presentation, the prognosis of PIGCH is unpredictable. Shetty et al summarized 5 drugs and 1 herbal medicine currently associated with PIGCH and their respective prognoses: Methotrexate (mild hepatitis), Chlorpromazine (mild hepatitis), Clometacine (acute liver failure), Amoxicillin + Clavulanate (chronic hepatitis with acute decompensation), Doxycycline (acute liver failure), and ISABGOL also known as Psyllium husk (mild hepatitis), a herbal remedy used for treating constipation [12] . Three out of the six drugs resulted in clinical improvement within 2-6 months either spontaneously or with the use of prednisone, while the other three led to liver failure.…”

Section: Assessmentmentioning

confidence: 99%

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Diclofenac-Induced Post Infantile Giant Cell Hepatitis

Venkataraman

Mohan

Conyers

et al. 2018

Journal of Gastroenterology and Hepatology Research

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unknown quantity of garlic tablets. Workup for hepatitis including infectious and toxic etiologies were ruled out. Serum IgG levels were elevated with increased globuin gap, and positive ANA titer of 640, anti-smooth muscle cell antibodies elevated at 41 units (moderate to strong positive > 30 units). Liver biopsy demonstrated submassive necrosis in the setting of post-infantile giant cell hepatitis. The differential diagnoses included idiopathic autoimmune hepatitis, viral or drug induced injury. Our diagnosis was drug induced autoimmune liver disease (DIALD), the etiology is likely due to Diclofenac given temporal relationship of drug intake and symptom onset, as well as a calculated Roussel Uclaf Causality Assessment Method (RUCAM) score of 6, signifying probable drug-induced liver disease. Patient was started on Prednisone and Azathioprine with complete resolution of clinical and laboratory findings within 5 months. DISCUSSION: The outcome of PIGCH is unpredictable, usually adopts a fulminant course leading to cirrhosis within months, however, a more benign course has also been described. PIGCH is triggered by three primary etiologies: drugs, infection, and autoimmune liver injury. This case highlights that giant cell hepatitis is a multifactorial condition that can be precipitated by drug use in the setting of autoimmune phenotype. Garlic and garlic extracts, through their antioxidant activities, have been reported to provide protection against free radical damage in the body. The innocuous course of PIGCH in our patient could possibly be due to concurrent consumption of garlic tablets. Further research is needed in this area.

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Giant-cell Hepatitis—Rare Entity in Adults

Cited by 7 publications

References 9 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

Erfolgreiche Therapie einer idiopathischen postinfantilen Riesenzellhepatitis mit einem Calcineurininhibitor

Diclofenac-Induced Post Infantile Giant Cell Hepatitis

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