Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome
Juvenile autoimmune hepatitis (JAIH) is severe immune-mediated necro-inflammatory disease of the liver with spontaneous progression to cirrhosis and liver failure if left untreated. The diagnosis is based on the combination of clinical, laboratory and histological findings. Prothrombin ratio is a us...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-08-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/13/17/2753 |
| _version_ | 1797582703877423104 |
|---|---|
| author | Silvia Nastasio Antonella Mosca Tommaso Alterio Marco Sciveres Giuseppe Maggiore |
| author_facet | Silvia Nastasio Antonella Mosca Tommaso Alterio Marco Sciveres Giuseppe Maggiore |
| author_sort | Silvia Nastasio |
| collection | DOAJ |
| description | Juvenile autoimmune hepatitis (JAIH) is severe immune-mediated necro-inflammatory disease of the liver with spontaneous progression to cirrhosis and liver failure if left untreated. The diagnosis is based on the combination of clinical, laboratory and histological findings. Prothrombin ratio is a useful prognostic factor to identify patients who will most likely require a liver transplant by adolescence or early adulthood. JAIH treatment consists of immune suppression and should be started promptly at diagnosis to halt inflammatory liver damage and ultimately prevent fibrosis and progression to end-stage liver disease. The risk of relapse is high especially in the setting of poor treatment compliance. Recent evidence however suggests that treatment discontinuation is possible after a prolonged period of normal aminotransferase activity without the need for liver biopsy prior to withdrawal. |
| first_indexed | 2024-03-10T23:26:16Z |
| format | Article |
| id | doaj.art-f0784dc650b54f22b62486cac05b5d2e |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-10T23:26:16Z |
| publishDate | 2023-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-f0784dc650b54f22b62486cac05b5d2e2023-11-19T07:59:07ZengMDPI AGDiagnostics2075-44182023-08-011317275310.3390/diagnostics13172753Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term OutcomeSilvia Nastasio0Antonella Mosca1Tommaso Alterio2Marco Sciveres3Giuseppe Maggiore4Division of Gastroenterology, Hepatology & Nutrition, Boston Children’s Hospital and Harvard Medical School, Boston, MA 02115, USAHepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyHepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyPediatric Department and Transplantation, ISMETT, 90133 Palermo, ItalyHepatogastroenterology, Rehabilitative Nutrition, Digestive Endoscopy and Liver Transplant Unit, ERN RARE LIVER, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, ItalyJuvenile autoimmune hepatitis (JAIH) is severe immune-mediated necro-inflammatory disease of the liver with spontaneous progression to cirrhosis and liver failure if left untreated. The diagnosis is based on the combination of clinical, laboratory and histological findings. Prothrombin ratio is a useful prognostic factor to identify patients who will most likely require a liver transplant by adolescence or early adulthood. JAIH treatment consists of immune suppression and should be started promptly at diagnosis to halt inflammatory liver damage and ultimately prevent fibrosis and progression to end-stage liver disease. The risk of relapse is high especially in the setting of poor treatment compliance. Recent evidence however suggests that treatment discontinuation is possible after a prolonged period of normal aminotransferase activity without the need for liver biopsy prior to withdrawal.https://www.mdpi.com/2075-4418/13/17/2753autoimmune hepatitisacute liver failureautoimmune liver diseaseactive chronic hepatitis liver transplantation |
| spellingShingle | Silvia Nastasio Antonella Mosca Tommaso Alterio Marco Sciveres Giuseppe Maggiore Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome Diagnostics autoimmune hepatitis acute liver failure autoimmune liver disease active chronic hepatitis liver transplantation |
| title | Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome |
| title_full | Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome |
| title_fullStr | Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome |
| title_full_unstemmed | Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome |
| title_short | Juvenile Autoimmune Hepatitis: Recent Advances in Diagnosis, Management and Long-Term Outcome |
| title_sort | juvenile autoimmune hepatitis recent advances in diagnosis management and long term outcome |
| topic | autoimmune hepatitis acute liver failure autoimmune liver disease active chronic hepatitis liver transplantation |
| url | https://www.mdpi.com/2075-4418/13/17/2753 |
| work_keys_str_mv | AT silvianastasio juvenileautoimmunehepatitisrecentadvancesindiagnosismanagementandlongtermoutcome AT antonellamosca juvenileautoimmunehepatitisrecentadvancesindiagnosismanagementandlongtermoutcome AT tommasoalterio juvenileautoimmunehepatitisrecentadvancesindiagnosismanagementandlongtermoutcome AT marcosciveres juvenileautoimmunehepatitisrecentadvancesindiagnosismanagementandlongtermoutcome AT giuseppemaggiore juvenileautoimmunehepatitisrecentadvancesindiagnosismanagementandlongtermoutcome |