University of South Florida Titusville, FL, United States
Pratik Patel, MD1, Christopher Albers, MD2, Nyingi Kemmer, MD, MSc, MPH2, Saurabh Agrawal, MD2 1University of South Florida, Titusville, FL; 2Tampa General Medical Group, Tampa, FL
Introduction: Type II Autoimmune Hepatitis (AIH) is an inflammatory condition that is not commonly diagnosed in the United States. While Type I AIH generally presents with a more indolent course, type II AIH can initially present with severe acute hepatitis in up to 40% of cases. Nonetheless, liver cirrhosis at the time of initial presentation is very rare for Type II AIH. We present a case of Type II AIH presenting with severe acute hepatitis with underlying liver cirrhosis.
Case Description/Methods: A previously healthy 19-year-old Hispanic female initially presented to an outside facility with jaundice. Initial blood work revealed ALT 1111, ALT 542, ALP 295, total bilirubin 12.9, and INR of 1.3. Liver disease workup was only positive for anti-liver kidney microsomal antibody type 1 [anti-LKM1]. Imaging revealed a nodular liver consistent with cirrhosis and an unremarkable biliary tree. Liver biopsy revealed a portosystemic gradient of 12-13 mmHg. Pathology was consistent with severe acute hepatitis with extensive parenchymal extinction and necrosis, moderate to severe mixed inflammation and pan-zonal hepatocyte loss.
A few weeks later, she developed ascites and was admitted at our facility for transplant evaluation. CT scan with liver protocol confirmed shrunken, nodular liver consistent with cirrhosis. She received 3 days of IV methylprednisolone and was transitioned to oral steroids as her liver function tests were improving. Transplant evaluation was initiated as admission MELD was 26 with coagulopathy, however she did not require transplantation given improvement and lack of encephalopathy. She was discharged home in stable condition on oral prednisone and close Hepatology follow up.
Discussion: Type I AIH has a more chronic presentation then Type I AIH with a greater incidence of cirrhosis at the time of diagnosis. Type II AIH presents with acute liver failure in up to 40% of patients, however, it is exceedingly rare to have developed cirrhosis at the time of initial diagnosis. Our patient was diagnosed with Type II AIH based on positivity to anti-LKM1 along with negative ASMA/ANA and elevated IgG. Type II AIH should be considered in children and young adults presenting with liver enzyme abnormalities. Evaluation for liver transplant should be initiated promptly, especially if there is evidence of coagulopathy or hepatic encephalopathy. Type II AIH has a high rate of relapse, and transplant evaluation despite initial improvement may improve outcomes and prevent delays in case of future decompensation.
Disclosures: Pratik Patel indicated no relevant financial relationships. Christopher Albers indicated no relevant financial relationships. Nyingi Kemmer indicated no relevant financial relationships. Saurabh Agrawal indicated no relevant financial relationships.
Pratik Patel, MD1, Christopher Albers, MD2, Nyingi Kemmer, MD, MSc, MPH2, Saurabh Agrawal, MD2. P1910 - Cirrhosis at Initial Presentation of Type II Autoimmune Hepatitis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.