Co-existence of Sickle Hepatopathy with Autoimmune Hepatitis: More than a Coincidence – A Case Report

Abstract

Sickle Cell Hepatopathy is a multifactorial liver disease that can have an impact on half of patients with Sickle Cell Disease (SCD), with a cryptic and puzzling connection appearing to be associating Sickle Cell Disease (SCD) with Autoimmune Hepatitis (AIH), repeatedly yielding diagnostic neglect and delay, bringing about disastrous consequences. This case introduces to you a 41-year-old single Saudi male known to have Sickle Cell Disease (SCD), who was accidentally diagnosed with advanced liver cirrhosis by pure chance during laparoscopic cholecystectomy. This study will draw attention to the diagnostic challenges concerning the overlap between sickle hepatopathy and Autoimmune Hepatitis (AIH), while sharing intriguing histopathological findings. Suggested theories that point out the unique relationship between the two entities are offered for a greater understanding of the complex pathophysiological process. Literature gaps are also discussed, owing to the absence of an agreed-upon recommended and safe therapeutic option for Autoimmune Hepatitis (AIH) in the context of Sickle Cell Disease (SCD) specifically, which forced starting the patient on the second line of treatment – Mycophenolate Mofetil (MMF). We learn from this case the value of early screening for liver disease in patients with Sickle Cell Disease (SCD) as well as the essential need for tailoring the therapeutic plan according to the type of patients when Autoimmune Hepatitis (AIH) is present.