Autoimmune Hepatitis Overlap with Primary Biliary Cirrhosis in an HIV Infected Patient

Abstract

42 year old Hispanic female with HIV on anti-retroviral therapy (ART) since 2009 and polyarthritis referred for evaluation of elevated LFTs. Her only complaint was mild itching of her body. She denied any use of herbal/ dietary supplements, alcohol abuse or illicit drug use. Her exam was unremarkable. Her LFTs were elevated- Alkaline phosphatase 410, AST 213, ALT 153, total bilirubin 0.5 mg/dl. Autoantibody testing showede elevated AMA at 78.6 and ANA titers 1:640. Viral hepatitis serologies including Hepatitis B surface antigen and Hepatitis C RNA PCR were negative. Her iron panel and ceruloplasmin levels were also normal. She was found to have hypergammaglobulinemia with elevated serum IgG levels of 4,117mg/dl (normal IgG less than 1730 mg/dl) and IGg subclass 4 elevated at 264 mg/dl (normal IgG < 86 mg/dl). US Abdomen showed multiple gallstones and gallbladder polyps, CBD was 2 mm and spleen size was normal. Percutaneous liver biopsy was performed and showed highly active portal and lobular hepatitis with evidence of bile duct inflammation and damage consistent with overlap syndrome of autoimmune hepatitis (AIH) with Primary biliary cirrhosis (PBC) and portal and periportal fibrosis with early bridging. Her medications included Hydroxychloroquine & Efavirenz-emtrictabine-tenofovir. Based on her liver biopsy results, patient was started on high dose prednisone 1mg/ kg and Azathioprine with improvement of her LFTs. She was continued on her ART throughout. ‘Overlap’ syndrome is a rare interface between AIH and PBC and is rarely reported in HIV infected adults. The common causes of elevated LFTs in HIV patients include infections and drug induced liver injury secondary to ART. Autoimmune disease in HIV although rare is an important differential while evaluating elevated LFTs in these patients. Unmasking of autoimmune disease is thought to be a manifestation of immune reconstitution with an era of highly effective ART in HIV patients. Our patient was on ART and had a CD4 count >600 suggesting a robust immune system. Her intial chronic liver disease workup suggested autoimmune disease and was confirmed histologically. Early liver biopsy can prevent ART interruption and its related risks. Standard treatment with immune suppressants appears to be effective & safe in HIV patients with high CD4 counts. To the best of our knowledge there is only one other previous case report of biopsy proven overlap syndrome with PBC/AIH in HIV patients.Figure 1Figure 2Figure 3