LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW.
• In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis and hepatic sequestration in the acute setting,...
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PAGEPress Publications
2023-10-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | http://www.mjhid.org/index.php/mjhid/article/view/5413 |
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| author | Kelvin Lynch Andrea Mega Andrea Piccin Massimo Daves Helen Fogarty |
| author_facet | Kelvin Lynch Andrea Mega Andrea Piccin Massimo Daves Helen Fogarty |
| author_sort | Kelvin Lynch |
| collection | DOAJ |
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• In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis and hepatic sequestration in the acute setting, and cholelithiasis and iron overload in the chronic setting. Autoimmune hepatitis (AIH) is a rare disease that appears to occur more commonly in the sickle cell disease (SCD) population than in the general population. There are many schools of thought as to why this is the case, including the phosphatidylserine hypothesis, the heme inflammatory hypothesis, the complement generation hypothesis, and the transfusion alloimmunization hypothesis.
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• Due to the natural history of the two illnesses, SCD is almost always diagnosed first in cases of dual pathology. Symptoms such as jaundice, fatigue, and abdominal pain are common in SCD, as are abnormal liver function tests (LFTs). These abnormalities, attributed to the other more frequent liver involvements in SCD, can lead to delays in AIH diagnosis in this population.
• Corticosteroids, sometimes with other immunosuppressive agents, such as azathioprine, are the cornerstone of acute AIH treatment (4). However, corticosteroid use in the SCD population has been shown to carry an increased risk of vaso-occlusive crises (1, 5), providing a treatment dilemma.
•
• The following is a review of AIH in the SCD population, where we explore the pathophysiology behind the association between the two disorders, discuss an approach to investigating abnormal LFTs in SCD, and examine treatment options in this population with co-existing diseases.
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| format | Article |
| id | doaj-art-34d6e151901d44fe81a67fe2a967c309 |
| institution | Kabale University |
| issn | 2035-3006 |
| language | English |
| publishDate | 2023-10-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-34d6e151901d44fe81a67fe2a967c3092025-01-02T23:54:24ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062023-10-0115110.4084/MJHID.2023.060LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. Kelvin Lynch0Andrea Mega1Andrea Piccin2Massimo Daves3Helen Fogarty4a:1:{s:5:"en_US";s:24:"Cork University Hospital";}Dept of Gastroenterology, Provincial Hospital of Bolzano (SABES-ASDAA), Bolzano, ItalyConsultant Haematologist, FRCPath, PhD NORTHERN IRELAND BLOOD TRANSFUSION SERVICE NIBTS, Belfast, UKDept of Laboratory Medicine, Provincial Hospital of Bolzano (SABES-ASDAA), Bolzano, ItalyDepartment of Haematology, Children’s HealtIrish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin • In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis and hepatic sequestration in the acute setting, and cholelithiasis and iron overload in the chronic setting. Autoimmune hepatitis (AIH) is a rare disease that appears to occur more commonly in the sickle cell disease (SCD) population than in the general population. There are many schools of thought as to why this is the case, including the phosphatidylserine hypothesis, the heme inflammatory hypothesis, the complement generation hypothesis, and the transfusion alloimmunization hypothesis. • • Due to the natural history of the two illnesses, SCD is almost always diagnosed first in cases of dual pathology. Symptoms such as jaundice, fatigue, and abdominal pain are common in SCD, as are abnormal liver function tests (LFTs). These abnormalities, attributed to the other more frequent liver involvements in SCD, can lead to delays in AIH diagnosis in this population. • Corticosteroids, sometimes with other immunosuppressive agents, such as azathioprine, are the cornerstone of acute AIH treatment (4). However, corticosteroid use in the SCD population has been shown to carry an increased risk of vaso-occlusive crises (1, 5), providing a treatment dilemma. • • The following is a review of AIH in the SCD population, where we explore the pathophysiology behind the association between the two disorders, discuss an approach to investigating abnormal LFTs in SCD, and examine treatment options in this population with co-existing diseases. http://www.mjhid.org/index.php/mjhid/article/view/5413Sickle CellAutoimmuneLiver DiseaseHepaititsSteroidsVaso-occlusive Crisis |
| spellingShingle | Kelvin Lynch Andrea Mega Andrea Piccin Massimo Daves Helen Fogarty LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. Mediterranean Journal of Hematology and Infectious Diseases Sickle Cell Autoimmune Liver Disease Hepaitits Steroids Vaso-occlusive Crisis |
| title | LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. |
| title_full | LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. |
| title_fullStr | LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. |
| title_full_unstemmed | LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. |
| title_short | LIVER DISEASE & SICKLE CELL DISEASE: AUTOIMMUNE HEPATITIS MORE THAN A COINCIDENCE; A SYSTEMATIC LITERATURE REVIEW. |
| title_sort | liver disease sickle cell disease autoimmune hepatitis more than a coincidence a systematic literature review |
| topic | Sickle Cell Autoimmune Liver Disease Hepaitits Steroids Vaso-occlusive Crisis |
| url | http://www.mjhid.org/index.php/mjhid/article/view/5413 |
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