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

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Pinelopi Arvaniti ◽  
Kalliopi Zachou ◽  
George K. Koukoulis ◽  
George N. Dalekos
Keyword(s):  
Liver Disease ◽  
Autoimmune Hepatitis ◽  
Giant Cell ◽  
Liver Diseases ◽  
Acute Liver Injury ◽  
Rescue Therapy ◽  
Primary Myelofibrosis ◽  
Life Saving ◽  
History Of ◽  
Giant Cell Hepatitis

Giant cell hepatitis (GCH) is commonly reported in neonatal and infantile liver diseases but rarely in adults where the term postinfantile GCH (PIGCH) is used. PIGCH is associated with many diseases, including drugs toxicity, viruses, and autoimmune liver diseases, with autoimmune hepatitis (AIH) being the most prevalent. We present a case of PIGCH in a 76-year-old female without known history of liver disease who suffered from an acute severe episode of hepatitis. After careful exclusion of other hepatitis causes by imaging, virological, immunological, and microbiological investigations, a diagnosis of acute severe AIH (AS-AIH) was established. The patient was started on corticosteroids but she did not respond and died 3 days later because of advanced acute liver failure. Postmortem liver biopsy showed typical PIGCH lesions. Physicians must keep this catastrophic entity in mind in cases of unexplained acute liver injury as, contrary to our case, prompt rescue therapy with corticosteroids may be life-saving.

Postinfantile giant cell hepatitis in the setting of autoimmune hepatitis: exclusively a histological pattern or a prognosis predictor?

2021 ◽  
Vol 14 (7) ◽  
pp. e243660
Author(s):  
Carolina Teles ◽  
Rui Santos ◽  
Carlos Dias Silva ◽  
Teresa Vaio
Keyword(s):  
Liver Disease ◽  
Liver Failure ◽  
Autoimmune Hepatitis ◽  
Immunosuppressive Therapy ◽  
Giant Cell ◽  
Viral Infections ◽  
Chronic Liver ◽  
Close Monitoring ◽  
Histological Pattern ◽  
Giant Cell Hepatitis

Autoimmune hepatitis (AIH) is a rare chronic liver disease with a non-specific clinical presentation. Its physiopathology is not fully understood and, if untreated, can progress to cirrhosis and even fulminant liver failure. Here, we describe a case of a 73-year-old patient with an 11-month history suggestive of liver disease, who was concomitantly diagnosed with AIH and the extremely rare postinfantile giant cell hepatitis (PIGCH). Despite standard immunosuppressive therapy, the patient presented a severe clinical course, culminating in acute-on-chronic liver failure and death. This case reminds physicians of the importance of an early diagnosis, close monitoring and timely treatment of AIH. It also highlights the significant role in prognosis of the specific histological pattern of PIGCH, which has been mainly associated with a serious clinical outcome and unpredictable response to immunosuppressive therapy. Triggers of both AIH and PIGCH, such as viral infections, must be excluded, given their treatment implications.

Primary Biliary Cirrhosis

2015 ◽  
Author(s):  
Daniel S. Pratt ◽  
Lindsay Y. King
Keyword(s):  
Differential Diagnosis ◽  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Liver Diseases ◽  
American Association ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Common Cause ◽  
History Of

Primary biliary cirrhosis (PBC) is a progressive autoimmune disease of the liver. It is the most common cause of chronic intrahepatic cholestatic liver disease in adults. This review addresses the epidemiology, etiology and genetics, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, complications, and prognosis of PBC. Figures show the pathogenesis and natural history of PBC and histologic features of the four stages of PBC. Tables list diagnostic criteria for PBC via the American Association for the Study of Liver Diseases, differential diagnosis for PBC, medications used to treat PBC, secondary therapy for PBC, and follow-up of patients with PBC. This review contains 2 highly rendered figures, 5 tables, and 45 references.

Autoimmunity in liver disease

2019 ◽  
pp. 245-252
Author(s):  
Gavin Spickett
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Autoimmune Hepatitis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Biliary Cirrhosis

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of liver diseases. Primary biliary cirrhosis, autoimmune hepatitis, and primary sclerosing cholangitis are described.

Post-infantile giant cell hepatitis in patients with primary sclerosing cholangitis and autoimmune hepatitis

2008 ◽  
Vol 16 (4) ◽  
pp. 274-282 ◽  
Author(s):  
U. Protzer ◽  
H. P. Dienes ◽  
L. Bianchi ◽  
A. W. Lohse ◽  
I. Helmreich-Becker ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Giant Cell ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Giant Cell Hepatitis

Syncytial giant-cell hepatitis due to autoimmune hepatitis type II (LKM1+) presenting as subfulminant hepatitis

2000 ◽  
Vol 95 (3) ◽  
pp. 799-801 ◽  
Author(s):  
Ziv Ben-Ari ◽  
Effrat Broida ◽  
Yehudit Monselise ◽  
Anna Kazatsker ◽  
Jacob Baruch ◽  
...  
Keyword(s):  
Autoimmune Hepatitis ◽  
Giant Cell ◽  
Type Ii ◽  
Giant Cell Hepatitis

Post-Infantile Giant Cell Hepatitis Associated with Autoimmune Hepatitis and Polyarteritis Nodosa

2002 ◽  
Vol 37 (1) ◽  
pp. 120-123 ◽  
Author(s):  
J. Koskinas ◽  
M. Deutsch ◽  
C. Papaioannou ◽  
G. Kafiri ◽  
S. Hadziyannis
Keyword(s):  
Autoimmune Hepatitis ◽  
Giant Cell ◽  
Polyarteritis Nodosa ◽  
Giant Cell Hepatitis
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