D0563 - Liver Function Abnormalities in Adult Onset Still's Disease: A Case Report

Tanisha Kalra, MD, Mohammad Almeqdadi, MD, Savanna Thor, DO, MPH
SUNY Downstate Health Science University, Brooklyn, NY

Introduction: Adult Onset Still’s disease(AOSD) has an estimated incidence of 0.16-0.4 per 100,000 people¹. Hence, liver dysfunction due to AOSD is a rare entity with a wide spectrum from hepatomegaly and elevated transaminases to fulminant hepatic failure. Delayed diagnosis can lead to life-threatening liver failure. We report a case of liver dysfunction due to AOSD diagnosed after extensive evaluation.

Case Description/Methods: A 27-year-old African-American woman presented to the emergency department with 2 weeks of fever, chills, myalgia, non-bloody watery diarrhea, shortness of breath, and pleuritic chest pain, which started after a recent trip to Haiti. On exam, she was febrile (103 F), tachycardic, hypoxic, with tender bilateral axillary and inguinal lymphadenopathy. Treatment with antibiotics, methylprednisolone, and anti-tubercular therapy (RIPE) was initiated. She required intubation and vasopressor support due to worsened respiratory distress. Her liver enzymes were noted to be trending up after admission. RIPE therapy was modified but transaminases continued to rise. Extensive infectious and rheumatological workup was negative. Work-up for chronic liver disease was negative as well (table 1). A sonogram with doppler and MRCP showed normal hepatobiliary anatomy. Inguinal lymph node biopsy showed reactive hyperplastic patterns. A liver biopsy showed few nonspecific small lobular foci of spotty inflammation, necrosis and ballooning degeneration of hepatocytes. The patient was diagnosed with AOSD based on Yamaguchi criteria since she met 3 major and 3 minor criteria. Given lack of improvement with methylprednisolone, interleukin-1β inhibitor canakinumab was started. Inflammatory markers and liver enzymes improved significantly and she was discharged.

Discussion: AOSD is a rare disease with nonspecific signs and symptoms. Liver function abnormalities are the most common manifestation in AOSD. Liver involvement can occur in the absence of other features of the disease and can delay diagnosis. Diagnosis is based on clinical criteria. Treatment is largely based on expert opinion since controlled trials are lacking. The most effective treatment is corticosteroids and biologic therapy. This case highlights the need for a high index of suspicion for AOSD when abnormal liver enzymes are seen in the context of nonspecific signs and symptoms.

References
1. Gerfaud-Valentin, Mathieu, Yvan Jamilloux, Jean Iwaz, and Pascal Sève. 2014. Adult-onset Still's disease, Autoimmunity Reviews, 13: 708-22.



Disclosures:


Tanisha Kalra, MD, Mohammad Almeqdadi, MD, Savanna Thor, DO, MPH. D0563 - Liver Function Abnormalities in Adult Onset Still's Disease: A Case Report, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.