University of Pennsylvania Health System Philadelphia, PA, United States
Stephen Schwartz, DO, Frederick Nunes, MD, K. Rajender Reddy, MD, Rahul Chaudhari, MD, MRCP, Yan Feng, MD, PhD, Maureen Whitsett, MD, Zhaohai Yang, MD, PhD, Katherine Murphy, MD University of Pennsylvania Health System, Philadelphia, PA
Introduction: Early experience in the pandemic in northern Italy has proven that patients with preexisting autoimmune liver disease (AILD) are not at higher risk for COVID-19 complications and mortality compared to the general population; however, the association between SARS-CoV-2 and hepatic decompensation remains unclear. We report a case of primary biliary cholangitis (PBC)/autoimmune hepatitis (AIH) overlap syndrome in which COVID-19 led to increased liver enzyme derangements.
Case Description/Methods: The patient is a 54 year-old woman with a medical history significant for hypertension, hyperlipidemia, Type 2 diabetes, and a recent transformation of PBC to PBC/AIH overlap syndrome, who presented to our institution with COVID-19. Prior to presentation, the patient’s prednisone had been tapered to 5 mg daily and she remained on MMF, sulfamethoxazole-trimethoprim prophylaxis, and ursodiol. On presentation, the patient was febrile to 101.5°F but otherwise hemodynamically stable. The patient appeared jaundiced with conjunctival icterus. Initial labs revealed AST (680 IU/L), ALT (391 IU/L), total bilirubin (1.9 mg/dL, direct 1.3 mg/dL), ALP (439 IU/L). Abdominal doppler US and MRCP were unremarkable and further infectious etiologies were excluded. Under the assumption that she had a flare of her PBC/AIH in addition to COVID-19 infection, her prednisone was increased to 20 mg and MMF was held while she received hydroxychloroquine and antibiotics. She did not require supplemental oxygen and at the time of discharge her aminotransferases improved: AST/ALT (376/293 IU/L). She was discharged on prednisone 10 mg daily with resumption of MMF and ursodiol. After discharge, the patient underwent repeat liver biopsy which revealed a biliary pattern of injury, with moderate portal fibrosis and bile duct loss.
Discussion: In our patient with preexisting PBC/AIH, we hypothesize that COVID-19 induced a flare of the patient’s AILD but was not responsible for direct liver injury. The final biopsy of our patient evidenced preexisting PBC and progressive AIH. There is no steatosis, increased eosinophilic infiltrates, or other evidence of direct SARS-CoV-2 mediated injury or drug induced liver injury. Though SARS-CoV-2 likely did not cause direct liver injury in our patient with PBC/AIH overlap syndrome, it is plausible the virus precipitated an AIH flare as the patient’s aminotransferases improved with increased corticosteroids and treatment of underlying COVID-19.
Figure: Pathological changes in the liver biopsies before (A-C) and after (D-F) COVID-19 infection. The pre-COVID liver biopsy shows marked portal inflammation with abundant plasmacytic infiltration, bile duct damage, and severe interface activity with clusters of plasma cells (A). There is also marked lobular inflammation with clusters of plasma cells (B) (H&E stain, 400 x). Trichrome stain shows periportal fibrosis (C). The portal inflammation is much reduced in post-COVID biopsy (D), though bile damage remains, and there is bile duct loss in other portal tracts. The lobular inflammation is also reduced (E). Trichrome stain shows similar periportal fibrosis (F) (Trichrome stain, 200 x).
Disclosures: Stephen Schwartz indicated no relevant financial relationships. Frederick Nunes indicated no relevant financial relationships. K. Rajender Reddy indicated no relevant financial relationships. Rahul Chaudhari indicated no relevant financial relationships. Yan Feng indicated no relevant financial relationships. Maureen Whitsett indicated no relevant financial relationships. Zhaohai Yang indicated no relevant financial relationships. Katherine Murphy indicated no relevant financial relationships.
Stephen Schwartz, DO, Frederick Nunes, MD, K. Rajender Reddy, MD, Rahul Chaudhari, MD, MRCP, Yan Feng, MD, PhD, Maureen Whitsett, MD, Zhaohai Yang, MD, PhD, Katherine Murphy, MD. P2939 - An Interplay Between COVID-19 and Primary Biliary Cholangitis/Autoimmune Hepatitis Overlap Syndrome, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.