TY - JOUR
T1 - Autoimmune Liver Disease in Patients with Sickle Cell Disease
AU - Waisbourd-Zinman, Orith
AU - Frenklak, Rachel
AU - Hakakian, Odelia
AU - Hilmara, Didja
AU - Lin, Henry
N1 - Publisher Copyright:
© 2021 Lippincott Williams and Wilkins. All rights reserved.
PY - 2021/10/1
Y1 - 2021/10/1
N2 - Objective: Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/or sclerosing cholangitis (SC)-together, autoimmune liver disease (AILD). We aimed to better understand the relationship of these diseases in pediatric patients. Materials and Methods: A retrospective analysis of patients with SCD and AILD at the Children's Hospital of Philadelphia (January 2008 to August 2015). Results: Seven patients, ages 8 to 23 years (3 males), were identified. Three had AIH, 2 SC, and 2 AIH/SC overlap, known as autoimmune SC. All patients with AIH treated with azathioprine significantly improved their liver enzymes. One patient with SC and inflammatory bowel disease underwent successful bone marrow transplant. Two SC patients died from SCD complications. Conclusions: In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD.
AB - Objective: Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/or sclerosing cholangitis (SC)-together, autoimmune liver disease (AILD). We aimed to better understand the relationship of these diseases in pediatric patients. Materials and Methods: A retrospective analysis of patients with SCD and AILD at the Children's Hospital of Philadelphia (January 2008 to August 2015). Results: Seven patients, ages 8 to 23 years (3 males), were identified. Three had AIH, 2 SC, and 2 AIH/SC overlap, known as autoimmune SC. All patients with AIH treated with azathioprine significantly improved their liver enzymes. One patient with SC and inflammatory bowel disease underwent successful bone marrow transplant. Two SC patients died from SCD complications. Conclusions: In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD.
KW - autoimmune hepatitis
KW - autoimmune liver disease
KW - pediatric hematology
KW - sclerosing cholangitis
KW - sickle cell disease
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U2 - 10.1097/MPH.0000000000001985
DO - 10.1097/MPH.0000000000001985
M3 - Article
C2 - 33181587
AN - SCOPUS:85096650063
SN - 1077-4114
VL - 43
SP - 254
EP - 257
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 7
ER -