Kaiser Permanente Los Angeles Medical Center Los Angeles, CA, United States
Andy Tien, MD, MS, Tamira Robinson, ACNP-BC, Amandeep Sahota, MD, MS Kaiser Permanente Los Angeles Medical Center, Los Angeles, CA
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome which can lead to nonspecific hepatitis and fulminant hepatic failure. Early recognition and treatment are critical to survival. We present a case of a young female with discoid lupus and suspected autoimmune hepatitis (AIH) who was found to have a challenging case of refractory HLH.
Case Description/Methods: A 21-year-old female with history of discoid lupus on hydroxychloroquine, cannabis hyperemesis syndrome, and morbid obesity was recently admitted to an outside hospital for shock of unclear etiology presumed to be due to culture-negative sepsis. At that time, liver biopsy was performed for persistently elevated liver enzymes (peak ALT 533, AST 1327, AP 596, total bilirubin 5.8) which showed severe chronic active hepatitis (grade 3, stage 2) with interface hepatitis suggestive of AIH, acute viral hepatitis, and/or drug-induced liver injury. The biopsy also noted moderate increase in sinusoidal Kupffer cell iron which raised a remote suspicion of HLH. She was discharged on prednisone 20mg oral daily for likely autoimmune hepatitis. During a follow-up exam, she was found to be fatigued, hypotensive, and had persistently elevated liver enzymes despite prednisone. She was therefore admitted to our hospital. Infectious work-up was negative. Patient’s ferritin was >18,000 and along with her clinical picture, HLH was diagnosed. She was started on treatment with pulse steroids, mycophenylate mofetil, anakinra, and plasma exchange without clear improvement. Repeat liver biopsy showed bridging necrosis/fibrosis and hemophagocytosis with CD68 and CD31 immunostains which confirmed HLH. She was started on ruxolitinib without improvement and etoposide which was stopped due to neutropenia. She was then started on emapalumab-lzsg. Hospital course was complicated by cytomegalovirus viremia, rectal bleeding from cecal ulcer, septic shock, and cardiomyopathy. Patient clinically improved on emapalumab-lzsg with improved inflammatory markers and normalization of liver enzymes.
Discussion: Our case highlights the importance of keeping a broad differential for acute hepatitis. While AIH is often suspected in patients with rheumatologic disorders, other disorders such as HLH should be considered in a patient with elevated liver enzymes despite treatment. HLH is challenging to treat and alternative treatment options must be explored when patients are refractory to therapy.
Disclosures: Andy Tien indicated no relevant financial relationships. Tamira Robinson indicated no relevant financial relationships. Amandeep Sahota indicated no relevant financial relationships.
Andy Tien, MD, MS, Tamira Robinson, ACNP-BC, Amandeep Sahota, MD, MS. P0818 - Acute Hepatitis in a Young Female with Lupus: It’s Not Always What You Think, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.