Purpose: We wish to alert clinicians to the possible association between Sickle cell anemia (SCA) and autoimmune liver disease (AIL).
Methods: AIL was diagnosed by serologic, histologic and/or cholangiographic studies in patients with known SCA.
Results: Three schoolaged children with SCA were investigated for increased levels of transaminases and/or worsening jaundice. All were diagnosed to have AIL. Two patients had autoimmune hepatitis and have responded to immunosuppressive therapy, and I had primary sclerosing cholangitis and is stable with therapy.
Conclusions: Autoimmune hepatitis and sclerosing cholangitis are rare liver disorders of childhood, but may occur with greater frequency in patients with SCA. Early diagnosis and treatment is essential to prevent progression to liver cirrhosis. This association may provide a clue to the etiology of AIL and warrants further investigation.
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