IgG4-related autoimmune hepatitis with pathological characteristics of giant-cell hepatitis: A case report

2020 ◽  
Author(s):  
Tan You Wen ◽  
Jianhui Sheng ◽  
Xingbei Zhou
Keyword(s):  
Liver Function ◽  
Autoimmune Hepatitis ◽  
Giant Cell ◽  
Hepatitis A ◽  
Plasma Cells ◽  
Fibrous Tissue ◽  
Tissue Formation ◽  
Case Presentation ◽  
Portal Area ◽  
Giant Cell Hepatitis

Abstract Background ：Immunoglobulin (Ig)G4-positive plasma cells are thought to be present in some cases of autoimmune hepatitis (AIH), and giant-cell hepatitis (GCH) or synchronous giant hepatocytes are rare in some patients with AIH. Case presentation : A 68-year-old woman was hospitalized with asthenia, poor appetite, and yellow urine for more than 20 days. accompanied by obvious abnormal liver function, elevated IgG and IgG4 levels. Liver biopsy was performed, and the pathology showed extensive proliferation of fibrous tissue, formation of pseudolobules, moderate and severe interfacial inflammation, bridging necrosis and fibrosis, edema of hepatocytes as rosette-forming cells, multinucleated hepatocytes in each portal area, extensive monocyte lymph infiltration, dense plasma cells in the portal area, and positive plasma cells stained by IgG4 (more than 10 per portal area). The pathological diagnosis was IgG4-related AIH and GCH. Methylprednisolone was administered (20 mg/day). Two weeks later, her liver function gradually improved, and the immune indexes began to decline. Conclusion: we report a case of cirrhosis caused by AIH, which has the characteristics of IgG4 correlation and GCH in the hepatic pathology. Whether there is an inevitable relationship between them remains unclear.

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.

scholarly journals Felty’s Syndrome with Chronic Hepatitis and Compatible Autoimmune Hepatitis: A Case Presentation

2005 ◽  
Vol 44 (4) ◽  
pp. 335-341 ◽  
Author(s):  
Kaori SEMA ◽  
Masami TAKEI ◽  
Kumi UENOGAWA ◽  
Akira HORIKOSHI ◽  
Yoshifumi HOSOKAWA ◽  
...  
Keyword(s):  
Chronic Hepatitis ◽  
Autoimmune Hepatitis ◽  
Hepatitis A ◽  
Case Presentation ◽  
Felty's Syndrome ◽  
Felty’S Syndrome

scholarly journals Giant Cell Hepatitis – A Rare Association with Connective Tissue Disease

2019 ◽  
Vol 30 (4) ◽  
pp. 224
Author(s):  
Maria Rauf ◽  
Sambit Sen ◽  
Adam Levene ◽  
Muhammad K. Nisar
Keyword(s):  
Connective Tissue ◽  
Giant Cell ◽  
Connective Tissue Disease ◽  
Hepatitis A ◽  
Rare Association ◽  
Giant Cell Hepatitis

scholarly journals A case of acute hepatitis A histologically proven as giant cell hepatitis

Kanzo ◽  
2005 ◽  
Vol 46 (11) ◽  
pp. 641-646 ◽  
Author(s):  
Makoto OKADA ◽  
Hayato SANEFUJI ◽  
Jiro WATANABE ◽  
Takeaki SATOH ◽  
Makoto NAKAMUTA ◽  
...  
Keyword(s):  
Acute Hepatitis ◽  
Giant Cell ◽  
Hepatitis A ◽  
Acute Hepatitis A ◽  
Giant Cell Hepatitis

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.

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