P3899 - Hepatitis E Virus-Associated Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome Complicated by Suspected Autoimmune Myopathy

Dauris Rosario Lora, MD, Joshua Moran, MD, Costica Aloman, MD
Rush University Medical Center, Chicago, IL

Introduction: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are autoimmune liver diseases that may occur together as part of an overlap AIH-PBC syndrome or in conjunction with other autoimmune hepatic diseases such as primary sclerosing cholangitis. AIH-PBC may also be associated with extrahepatic autoimmune diseases, such as autoimmune thyroid diseases, Sjögren syndrome, inflammatory bowel disease, rheumatoid arthritis, and lupus.

Case Description/Methods: A 71 year old woman with a history of hypertension, hyperlipidemia, and diabetes presented to the emergency department with several days of muscle cramps, fatigue, and upper extremity weakness. Initial laboratories demonstrated WBC 13.02, Hemoglobin 11.8 g/dl, Creatinine 3.59 mg/dl, Total bilirubin 2.3 mg/dl, ALP 422 U/L, AST 356 IU/L, ALT 449 IU/L, and CK level 10,349 U/L. The patient was admitted and started on IV fluids and subsequently her CK improved and kidney function normalized, with resolution of her symptoms. Her rhabdomyolysis was initially attributed to long term statin use, however HMCGR antibody was negative. Anti-smooth muscle, antimitochondrial antibodies and Hepatitis E virus IgG were positive. Liver biopsy demonstrated lymphoplasmacytic infiltrate, occasional hepatocyte rosetting, and periportal fibrosis, compatible with autoimmune hepatitis (AIH), as well as ductal injury and ductular reaction present, suggestive of concurrent primary biliary cholangitis (PBC). Due to concern for an autoimmune etiology of the patient’s myopathy, EMG was performed but was nonspecific: a follow up muscle biopsy was inconclusive but suggested a possible underlying immune mediated necrotizing myopathy. The patient was treated with ursodiol, corticosteroids, and azathioprine: within two months her liver enzymes had normalized and after one year she remained in biochemical remission.

Discussion: AIH-PBC overlap syndrome is a constellation of autoimmune diseases, often associated with extrahepatic autoimmunity. Hepatitis E virus seropositivity has been correlated with autoimmune liver disease and is theorized to be a potential trigger of various autoimmune diseases, including myositis. While this patient’s muscle biopsy was not definitive for autoimmune myositis, the simultaneous onset of AIH, PBC, and myositis suggest a unifying autoimmune trigger resulting in the development of all three. In all patients with autoimmune liver disease, it is important to consider autoimmune etiologies when evaluating both hepatic and extrahepatic conditions.


Disclosures:


Dauris Rosario Lora, MD, Joshua Moran, MD, Costica Aloman, MD. P3899 - Hepatitis E Virus-Associated Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome Complicated by Suspected Autoimmune Myopathy, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.