Successful Treatment of Rapid Onset, Symptomatic de novo Non-alcoholic Steatohepatitis Following Liver Transplantation: A Case Report

Abstract

A 45 year old female with a body mass index (BMI) of 24 underwent successful liver transplantation (LT) for alcoholic cirrhosis using a donor liver from an obese woman with microvesicular steatosis (80%) and minimal macrovesicular steatosis (5-10%) on liver biopsy. Ascites and hepatosplenomegaly developed soon after LT with progressive increase of serum alkaline phosphatase to 1340 IU/L while aspartate aminotransferase (AST), and alanine transaminase (ALT), and total bilirubin remained normal. Imaging showed marked hepatomegaly, extensive fatty infiltration of the liver, and compression of the hepatic veins with narrowing of the intrahepatic inferior vena cava (IVC). Liver biopsy on post-operative day 39 revealed 90-100% macrovesicular steatosis, steatohepatitis, and portal fibrosis. A hepatic venogram showed a 10 cm segment of intrahepatic IVC stenosis that was stented, improving portal venous pressure measurements. However, portal hypertension requiring diuretic therapy and multiple paracenteses remained. By 3 months after LT, her liver had grown to 22 cm, transaminases increased 2-4 times the upper limit of normal with a 2:1 AST to ALT ratio. Liver biopsy at post-LT day 82 showed no change in steatosis and steatohepatitis despite corticosteroid withdrawal and interval periportal and perisinusoidal fibrosis. 12 weeks after LT, the patient was found to have low apolipoprotein B (65 mg/dL), high-density lipoprotein (HDL) (<10 mg/dL), low-density lipoproteins (LDL) (9 mg/dL), and total cholesterol (<50 mg/dL) levels. Therapy was started for NASH with high dose (800 IU daily) vitamin E and pioglitazone 15 mg daily, and she received topical vegetable oil and oral essential fatty acid supplements. Liver enzymes normalized after 3 months and her lipid profile improved markedly (HDL 27 mg/dL, total cholesterol 128 mg/dL), with progressive decrease in liver size and resolution of ascites after 5 months of therapy. At 2 years post-LT, the liver enzymes remain normal and lipids have normalized. ( J CLIN EXP HEPATOL 2013;3:70-74) R ecurrent non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) are known complications following orthotopic liver transplantation (LT), but rarely occur in patients without features of the metabolic syndrome. We present a case of rapidly progressive de novo NASH leading to the development of portal hypertension soon after LT. Treatment of NASH and essential fatty acid deficiency (EFAD) was associated with marked clinical, biochemical, and radiographic improvement. CASE REPORTOur patient is a 45 year old Caucasian female with a pretransplant BMI of 24 who was transplanted for biopsy proven alcoholic cirrhosis. Her MODEL FOR END-STAGE LIVER DISEASE (MELD) at transplantation was 42; she was very ill, admitted to the ICU and suitable organs for transplantation at the time were limited. The donor was a 38 year old female with a BMI of 38 who died of head trauma. A biopsy of the donor liver showed marked microvesicular steatosis (80%) and minimal macrovesicular steatos...

Non‐alcoholic fatty liver disease following liver transplantation: a clinical review

“…Due to conflicting data and evidence suggesting an increase in cardiovascular mortality associated with vitamin E, it is currently not recommended for the treatment of NASH in the diabetic population . However, the use of vitamin E in the post‐transplant setting has been successfully utilized in combination with pioglitazone in a recent case report of a patient with de novo NASH .…”

Hepatobiliary Quiz-6 (2013)

“…34 There is a case report of use of metformin and pioglitazone with biochemical improvement over five months of therapy. 35 6. Correct answers: 1, 4 and 5…”