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Teker Düztaş D, Öztürk H, Eğritaş Gürkan Ö, Esendağlı G, Sarı S, Dalgıç B, Dalgıç A. Overview of the Etiology, Pathology, and Prognosis of Giant Cell Hepatitis. EXP CLIN TRANSPLANT 2024; 22:106-110. [PMID: 39498930 DOI: 10.6002/ect.pedsymp2024.o30] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2024]
Abstract
OBJECTIVES Giant cell hepatitis is an important diagnostic consideration in early childhood, especially for patients who present with jaundice. Different diseases may play a role in their etiology. In this study, we presented pediatric patients in our center diagnosed with giant cell hepatitis. MATERIALS AND METHODS Liver biopsies performed in our hospital between January 2010 and December 2023 were retrospectively evaluated, and biopsies with histological giant cell formation were included in the study. Demographic, clinical, and histopathological characteristics of the patients were obtained from the medical records. RESULTS Our study included 31 cases of giant cell hepatitis. Twenty-four (90.3%) of the patients were male, and the median age was 4 months (range, 1-148 mo). The most common clinical findings were jaundice (93.5%). Among the 31 patients, idiopathic giant cell hepatitis was observed in 13 cases (41.9%), and etiology was elucidated in 18 patients. The most common diagnoses in these patients were progressive familial intrahepatic cholestasis, biliary atresia, and autoimmune hepatitis (22.6%, 19.4%, and 9.7%, respectively). The most common accompanying histopathologic findings were canalicular cholestasis (87.1%), lobular cholestasis (54.8%), and pericellular fibrosis (35.5%). Liver transplant was required in 10 patients (32.3%). Nine patients (29%) died from various reasons. Although follow-up of 13 patients (41.9%) are ongoing, 9 patients (29%) are no longer being followed. CONCLUSIONS Few studies in the pediatric age group on giant cell hepatitis have been conducted in the literature. Similar to our series, etiology could not be specified in most of the patients in these reports. Additional research is needed to improve our understanding of the disease process, uncover any other potential causes, and create specific treatment options.
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Affiliation(s)
- Demet Teker Düztaş
- From the Department of Pediatric Gastroenterology, Gazi University School of Medicine, Ankara, Turkey
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Martinez-Moad M, Wunderlich G, Diep D, Vasudevan A, Russell A, Janitz T, Aluri B, Oviedo A, Mysore Rangaraju A. Postinfantile Giant Cell Hepatitis Secondary to Rheumatoid Arthritis. ACG Case Rep J 2023; 10:e01006. [PMID: 37091202 PMCID: PMC10115553 DOI: 10.14309/crj.0000000000001006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2022] [Accepted: 02/03/2023] [Indexed: 04/25/2023] Open
Abstract
Postinfantile giant cell hepatitis (PIGCH), also known as syncytial giant cell hepatitis, continues to be a poorly defined and rare disease presentation in the adult population. Although a common finding in neonates, there is limited literature on the disease process, causes, and treatment success of PIGCH in adults. A strong association between autoimmune disorders and PIGCH, considerably so in the case of autoimmune hepatitis, has been established. However, there have been limited to no reports of PIGCH secondary to rheumatoid arthritis. Our clinical case aims to bring forth a vignette of PIGCH to spotlight this ill-defined disease in the adult population and highlight some of the proposed causes, treatments, and laboratory markers.
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Affiliation(s)
| | | | - Darlene Diep
- Burrell College of Osteopathic Medicine, Las Cruces, NM
| | | | | | - Tyler Janitz
- Burrell College of Osteopathic Medicine, Las Cruces, NM
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Jiao J, Zhang X. Post-infantile Giant Cell Hepatitis: A Literature Review and Meta-analysis. JOURNAL OF CLINICAL AND TRANSLATIONAL PATHOLOGY 2022; 2:100-107. [PMID: 37092012 PMCID: PMC10117396 DOI: 10.14218/jctp.2022.00016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
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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Affiliation(s)
| | - Xuchen Zhang
- Correspondence to: Xuchen Zhang, Department of Pathology, Yale University School of Medicine, 310 Cedar Street, PO Box 208023, New Haven, CT 06510, United States. Tel: +1 203-785-6010, Fax: +1 203-737-2922,
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Matta B, Cabello R, Rabinovitz M, Minervini M, Malik S. Post-infantile giant cell hepatitis: A single center’s experience over 25 years. World J Hepatol 2019; 11:752-760. [PMID: 31966907 PMCID: PMC6960295 DOI: 10.4254/wjh.v11.i12.752] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/14/2019] [Revised: 09/06/2019] [Accepted: 10/19/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Giant cell hepatitis in the adult population remains very poorly defined with only 100 case reports published in the literature over the last three decades.
AIM To present our center’s experience in an attempt to learn about the predisposing factors, outcomes and efficacy of proposed therapeutic interventions for giant cell hepatitis.
METHODS A retrospective chart review was conducted through the electronic records of the University of Pittsburgh Medical Center. We queried 36726 liver biopsy reports from January 1, 1991 to December 6, 2016. Our search yielded 50 patients who were identified as carrying a definite diagnosis of post-infantile giant cell hepatitis (PIGCH) by pathology. The data collected included demographic information, laboratory data (liver function tests, autoimmune markers) and transplant status. In order to better analyze patient characteristics and outcomes, subjects were separated into a non-transplant (native) liver group and a post-liver transplant (allograft) group.
RESULTS The incidence of PIGCH was approximately 0.14% of all biopsies queried in the 25-year period. The mean age was 48 years with 66% females. Liver function tests were classified as 38.2% cholestatic, 35.3% hepatocellular and 26.5% mixed. Autoimmune hepatitis was found to be the most prevalent predisposing factor leading to PIGCH constituting 32% of cases. Management consisted mainly of immunosuppression, viral targeted therapy, supportive care and in six cases liver transplantations.
CONCLUSION The diagnosis of PIGCH remains clinically challenging and requires a high index of suspicion as well as a thorough history, physical examination, serological workup and liver biopsy. Treatment of the underlying cause can result in clinical stability in a large number of cases.
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Affiliation(s)
- Bassem Matta
- Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Ricardo Cabello
- Department of Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States
| | - Mordechai Rabinovitz
- Department of Medicine, Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Marta Minervini
- Department of Pathology, University of Pittsburgh, Pittsburgh, PA 15213, United States
| | - Shahid Malik
- Department of Medicine, University of Pittsburgh, Division of Gastroenterology Hepatology and Nutrition, Pittsburgh, PA 15213, United States
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Asymptomatic giant cell hepatitis: a subtype of post-infantile giant cell hepatitis? Clin J Gastroenterol 2019; 12:367-371. [PMID: 30767175 DOI: 10.1007/s12328-019-00950-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2018] [Accepted: 02/09/2019] [Indexed: 01/23/2023]
Abstract
Giant cell hepatitis in adults is considered a rapidly progressive and life-threatening disease, but there are few descriptions of a prolonged disease course. A 36-year-old Japanese man was referred to our hospital for further evaluation of abnormal liver function test results. Although asymptomatic, he had undergone follow-up for 9 years with these abnormalities. Because the cause of liver injury was not identified despite extensive noninvasive examinations, the patient underwent needle biopsy. He was finally diagnosed with post-infantile giant cell hepatitis (PIGCH) based on the presence of small numbers of giant multinucleated hepatocytes scattered primarily around the portal area. Necroinflammatory changes were very mild in the portal tracts and hepatic parenchyma. According to the histological findings as well as the accepted international diagnostic scoring system for autoimmune hepatitis (AIH), which is closely related to PIGCH, AIH was unlikely, although antinuclear antibody was positive at a titer of 1:160. The present case may describe an unknown subtype of PIGCH, characterized by insidious disease onset and progression with concurrent, mildly active underlying hepatitis, which is in contrast with the well-documented aggressive nature of PIGCH.
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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. Case Reports Hepatol 2018; 2018:9793868. [PMID: 29713554 PMCID: PMC5866856 DOI: 10.1155/2018/9793868] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2017] [Revised: 01/11/2018] [Accepted: 02/11/2018] [Indexed: 01/28/2023] Open
Abstract
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.
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Bihari C, Rastogi A, Sarin SK. Postinfantile giant cell hepatitis: an etiological and prognostic perspective. HEPATITIS RESEARCH AND TREATMENT 2013; 2013:601290. [PMID: 23555054 PMCID: PMC3608114 DOI: 10.1155/2013/601290] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/16/2013] [Revised: 02/05/2013] [Accepted: 02/07/2013] [Indexed: 12/18/2022]
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.
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Affiliation(s)
- Chhagan Bihari
- Department of Pathology, Institute of Liver and Biliary Sciences (ILBS), D-1 Vasant Kunj, New Delhi 110070, India
| | - Archana Rastogi
- Department of Pathology, Institute of Liver and Biliary Sciences (ILBS), D-1 Vasant Kunj, New Delhi 110070, India
| | - Shiv Kumar Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences (ILBS), New Delhi 110070, India
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Anagnostopoulos GK, Margantinis G, Tsiakos S, Kostopoulos P, Grigoriadis K, Arvanitidis D. Postinfantile giant-cell hepatitis associated with ulcerative colitis and autoimmune hepatitis. J Gastroenterol Hepatol 2006; 21:1863-4. [PMID: 17074032 DOI: 10.1111/j.1440-1746.2006.03271.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/09/2022]
Abstract
Postinfantile giant cell hepatitis (PGCH) is rare. It is characterized by the presence of multinucleated giant cells in liver biopsy, and although it has been associated with several etiological agents, in many cases its etiology remains unclear. The case is presented herein of an adult woman with PGCH in the setting of ulcerative colitis and autoimmune hepatitis. The presence of autoimmune hepatitis in the patient is consistent and supports the autoimmune pathogenesis of PGCH in a subgroup of patients. Furthermore, this finding, along with others, suggests that PGCH may be included in the list of hepatic complications of inflammatory bowel disease.
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Kuntzen T, Friedrichs N, Fischer HP, Eis-Hübinger AM, Sauerbruch T, Spengler U. Postinfantile giant cell hepatitis with autoimmune features following a human herpesvirus 6-induced adverse drug reaction. Eur J Gastroenterol Hepatol 2005; 17:1131-4. [PMID: 16148562 DOI: 10.1097/00042737-200510000-00020] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Giant cell hepatitis (GCH) is frequently found in neonates, but rarely in adults. Diagnosis is made on the basis of the presence of hepatocellular multinucleate giant cells. The disease often takes a fulminant course with the development of cirrhosis within months, requiring transplantation or leading to death in a high percentage of cases. The aetiology and pathogenesis are unclear. Association with autoimmune disorders, viral infections and drug reactions, but also with congenital metabolic diseases such as alpha1-antitrypsin deficiency or haemosiderosis has been described. In some cases, no causative event has been found. Therefore, therapeutic options are controversially discussed. We present a patient with GCH with autoimmune features after a human herpesvirus 6 (HHV6)-induced adverse drug reaction, a combination that has not been reported before. High-dose immunosuppression led to dramatic improvements over the past year.
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Affiliation(s)
- Thomas Kuntzen
- Department of Internal Medicine I, Rheinische Friedrich-Wilhelms-Universitaet Bonn, Bonn, Germany
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Hoyas Pablos E, Lozano Gutiérrez F, Ramos Guerrero A, Miralles Sanchís EJ. [Giant cell hepatitis and chronic lymphocytic leukemia]. Med Clin (Barc) 2004; 122:477-8. [PMID: 15104964 DOI: 10.1016/s0025-7753(04)74277-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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