Abstract
BackgroundAutoimmune hepatitis–primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. Peripheral blood hypereosinophilia in conjunction with autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome has not been reported yet. Here we present a case of autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome with hypereosinophilia.Case presentationA 33-year-old Iranian man with the fatigue, jaundice, elevated liver enzymes and alkaline phosphatase, and hypereosinophilia was referred to our hospital. Viral and autoimmune hepatitis were excluded, and secondary workups for hypereosinophilia were all negative. Magnetic resonance cholangiopancreatography showed beaded appearance of intra- and extrahepatic biliary tree, and liver biopsy revealed interface hepatitis. Therefore, the diagnosis of autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome was made, and prednisolone, azathioprine, and ursodeoxycholic acid was initiated. His jaundice and peripheral blood eosinophilia resolved after 2 weeks, and he became completely asymptomatic.ConclusionEosinophils might contribute to the clinical presentation and disease complications.
Highlights
BackgroundAutoimmune hepatitis–primary sclerosing cholangitis (AIH-PSC) overlap syndrome is a clinical, biochemical, immunological, and histological feature of AIH with cholangiographic abnormalities characteristic of PSC [1]
Autoimmune hepatitis–primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography
Laboratory data demonstrated a pattern of hypereosinophilia and hepatocellular liver injury: alanine transaminase (ALT) 710 IU/L, aspartate transaminase (AST) 389 IU/L, alkaline phosphatase (ALP) 677 IU/L (40 130 IU/L), gamma glutamyl transferase (GGT) 167 U/L (8–61 U/L), total bilirubin 3.45 mg/dl (0.3–1.2 mg/dl), direct bilirubin 2.79 mg/ dl (0–0.3 mg/dl), prothrombin time (PT) 12.6 seconds, international normalized ratio (INR) 1.27 (0.8–1.2)
Summary
Autoimmune hepatitis–primary sclerosing cholangitis (AIH-PSC) overlap syndrome is a clinical, biochemical, immunological, and histological feature of AIH with cholangiographic abnormalities characteristic of PSC [1]. Case presentation A 33-year-old Iranian man was referred to our hospital because of fever, pruritus, jaundice, fatigue, dark urine, and pale stool for 3 months. He had no history of allergic disorder or recent travel. Infectious diseases were excluded by negative results for viral markers [hepatitis B surface antigen and hepatitis C virus antibody, hepatitis A virus antibody (IgM), and anti-human immunodeficiency virus (HIV) antibody], serum antibodies to helminthic parasites, serologic test for Fasciola hepatica, and stool examinations for parasites and protozoa were all negative. Prednisolone was gradually tapered to 5 mg during 2 months His jaundice and peripheral blood eosinophilia resolved after 2 weeks, and he became completely asymptomatic. MRCP of the patient after 6 months showed intrahepatic bile ducts, CHD, and CBD luminal irregularity without dilation (Fig. 3)
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