Autoimmune hepatitis with giant-cell transformation

Department of Gastroenterology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico.
Annals of hepatology: official journal of the Mexican Association of Hepatology (Impact Factor: 2.07). 01/2009; 8(1):68-70.
Source: PubMed


Giant-cell hepatitis (GCH), also known as postinfantile or syncytial giant cell hepatitis, is a frequent pattern of liver injury in the neonate, primarily seen in the first three months of life. Few cases in adults have been reported, some of them associated to autoimmune diseases such as autoimmune hepatitis.
We present a case of autoimmune hepatitis with giant cell transformation in a 39 year old male with polyarthritis. We discuss his clinical presentation and course. We made a review of the literature of agents associated to this diagnosis, the mechanisms involved in the formation of giant hepatocytes, the histological findings, clinical course, treatment options and prognosis of this rare entity.
In conclusion, the clinical course varies from normalization of hepatic histology to progression to cirrhosis and liver failure. The prognosis is dictated by the underlying liver disease and in the setting of autoimmune hepatitis the clinical course is usually severe with most of the patients progressing to cirrhosis. Prolonged treatment with corticosteroids and immunosuppressants is usually effective in rendering the cirrhosis inactive.

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Available from: Braulio Martínez-Benítez, Jul 27, 2015
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    • "1 Good (responded to immunossuppresion) [11] SLE 2 Moderate [2] [12] Autoimmune hemolytic anemia 3 P o o r [13] [14] [15] PSC + AIH 2 Moderate (rapid onset of cirrhosis) Good (mild hepatitis) [16] [17] AIH + polyarthritis 1 Moderate (early cirrhosis) [18] AIH + polyarteritis 1 Moderate (early cirrhosis) [19] AIH + UC 1 Moderate (early cirrhosis) [20] PBC 2 1 Poor, (liver failure) 1 moderate (early cirrhosis) [21] [22] AIH II 1 Poor (died) [23] Viral HAV 4 Poor (fatal liver failure) [1, 24–26] HEV 1 Good (mild hepatitis) [27] HBV 3 Good (1 acute hepatitis, 2 chronic hepatitis) [2] [7] HCV 22 Good (chronic hepatitis) [5, 28–30] EBV 3 Poor (fatal liver failure) [31] [32] [33] Paramyxoviruses 13 Poor (7 fatal liver failure, 6 died) [3] [26] [34] [35] HIV + HCV 2 Good (chronic hepatitis) [30] HIV 2 Good (chronic hepatitis) [36] HHV-6A 1 Good (chronic hepatitis) [27] CMV 1 Poor (acute liver failure with underlying Wilson's diseases) [37] Hypereosinophilia 3 2 Poor (liver failure) 1 Good [24] [32] [38] CLL 3 2 Poor (liver failure) 1 Good (responded to immunosuppression) [39] Posttransplant 10 Poor (recurrent disease, mostly required retransplant) [40] [41] [42] [43] Associations with three eosinophilia cases were reported. Two of them had fatal disease course [24] [38], and one had better outcome [32]. "
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    ABSTRACT: Giant cell hepatitis is common manifestation in pediatric liver diseases, but quite uncommon in adults, only about 100 cases reported in the English literature in the last two decades. Data for the present review were identified by a structured PubMed/MEDLINE search from 1963 to December 2012, using keywords postinfantile giant cell hepatitis (PIGCH), adult giant cell hepatitis, and syncytial giant cell hepatitis in adults and liver. We report a case of postinfantile giant cell hepatitis along with the review related to the etiology and respective outcome, as the literature in the last 20 years suggests. This condition is probably due to idiosyncratic or cytopathic response of individual to various hepatocytic stimuli. It is purely a histomorphological diagnosis and does not establish the etiology. Autoimmune liver diseases are most common etiology, in around 40% of cases, but various viruses, drugs, posttransplant condition, and other causes also have been reported. Prognosis depends upon the etiology. In this paper, we emphasized various causative factors of PIGCH and their respective outcome in patients affected by them. We also highlighted the possible pathogenesis and histopathological spectrum of this entity on the basis of description given in various studies and our limited experience of few cases.
    Full-text · Article · Mar 2013 · Hepatitis research and treatment
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    ABSTRACT: Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AIHA) is a rare disease occurring early in childhood. The exact cause of this illness is unknown and is assumed to be an autoimmune disorder. This disease often has a fatal outcome due to poor response to immunosuppression. In this paper, we report an 11-month-old male infant who presented initially with Coomb's positive autoimmune hemolytic anemia followed by histology-proven GCH and rapid progression to fulminant hepatic failure and death within two months from the onset of the disease despite aggressive immunosuppression. In addition, we review the available literature about the clinical presentation, diagnosis and therapeutic options of this rare disease.
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