P2475 - Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap Syndrome Presenting as Refractory Pruritus Requiring Molecular Adsorbent Recirculating System Therapy

Nathan DeRon, DO, Dylan Lopez, MD
Methodist Health System of Dallas, Dallas, TX

Introduction: Autoimmune hepatitis and primary biliary cholangitis (AIH-PBC) overlap syndrome is an uncommon diagnosis which presents with symptoms of cholestasis such as right upper quadrant abdominal pain and jaundice. Refractory pruritus requiring hospitalization is a rare complication of AIH-PBC. There is limited literature regarding therapeutic options and success rates for refractory pruritus in AIH-PBC. We present a case of AIH-PBC manifesting as refractory pruritus in a 35-year-old female requiring molecular adsorbent recirculating system (MARS) therapy for symptom management.

Case Description/Methods: The patient is a 35-year-old Caucasian female with a history of AIH-PBC, positive AMA, and positive ASMA who presented with generalized pruritus uncontrolled with her home cholestyramine and ursodiol therapy. The patient was previously seen as an outpatient and started on prednisone 40 mg daily for two weeks without significant relief. She reported recently increasing dark urine along and right upper quadrant abdominal discomfort. She reported being unable to sleep due to the pruritus and therefore was forced to quit her job due to daytime fatigue. On presentation, the patient’s total bilirubin was 20.8 mg/dL, mostly conjugated, increased from a baseline total bilirubin of approximately 4.2 mg/dL.

The patient was started on conservative multimodal management for pruritus with diphenhydramine, hydroxyzine, naltrexone, warm blankets, and heat packs without improvement. The patient then received a total of six days of plasmapheresis with mild relief which immediately worsened when discontinued. Therefore, the patient was transferred to the ICU for five days of MARS therapy. Her total bilirubin decreased to 4.5 mg/dL, and she experienced adequate relief of pruritus. The patient was transitioned out of the ICU and discharged in stable condition with follow-up for outpatient transplant evaluation.

Discussion: Pruritus is a feature of AIH-PBC which can become life-altering and cause significant distress to patients both clinically and socially. There are several case reports in which MARS is a useful therapy for refractory pruritus in the setting of AIH-PBC; however, there is minimal large population data supporting the use of MARS as a definitive therapy for refractory pruritus. We propose increasing the frequency of early MARS therapy in patients with refractory pruritus along with medical management as a bridge to transplantation while further investigation into this management technique is undertaken.


Disclosures:


Nathan DeRon, DO, Dylan Lopez, MD. P2475 - Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap Syndrome Presenting as Refractory Pruritus Requiring Molecular Adsorbent Recirculating System Therapy, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.