Abstract
Autoimmune sclerosing cholangitis is an overlap syndrome characterized by features of both autoimmune hepatitis and primary sclerosing cholangitis, the latter usually involving the large bile ducts. Autoimmune sclerosing cholangitis occurs more often in children than in adults and is frequently associated with inflammatory bowel disease, predominantly ulcerative colitis. We report a unique case of a 10-year-old Danish boy with severe small duct autoimmune sclerosing cholangitis and synchronic Crohn colitis. He was referred with a history of weight loss, abdominal pain, vomiting and diarrhea. Biochemical anomalies included elevated alanine aminotransferase, γ-glutamyl transferase and immunoglobulin G levels and the presence of smooth muscle antibodies and perinuclear antineutrophil cytoplasmic antibodies but normal alkaline phosphatase. Liver biopsy specimen revealed features of both autoimmune hepatitis and sclerosing cholangitis, the latter characterized by acute, hyperplastic and destructive inflammation – granulocytic epithelial lesion – of the small ducts. Magnetic resonance cholangiography was normal. Colonoscopic biopsies showed chronic inflammatory changes of the caecum and the ascending and transverse colon compatible with Crohn disease. Ursodeoxycholic acid and immunosuppressive treatment was initiated and within four weeks of treatment the general condition improved. Normalization of aminotransferase was seen at 21 weeks and γ-glutamyl transferase at 72 weeks after first admittance, while immunoglobulin G remained slightly increased.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1418596609736470
Highlights
Immune-mediated liver diseases fall into two broad categories, those with a hepatitic predominance: autoimmune hepatitis (AIH), and those with a predominance of cholestatic features: primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)
AIH is characterized by elevated serum aminotransferases, hypergammaglobulinaemia, circulating autoantibodies (primarily antinuclear antibodies (ANA) and smooth muscle cell antibodies (SMA)) and interface hepatitis on liver biopsy specimen, while markedly elevated serum alkaline
We present a case of a 10-year-old boy, diagnosed as autoimmune sclerosing cholangitis (AISC) with an unusual, severe small duct lesion and concomitant Crohn colitis
Summary
Immune-mediated liver diseases fall into two broad categories, those with a hepatitic predominance: autoimmune hepatitis (AIH), and those with a predominance of cholestatic features: primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). Colonoscopy showed scattered reddened mucosa with small aphtoid ulcers in the caecum and the ascending and transverse colon Biopsy specimens from these areas revealed active chronic inflammation compatible with CD (Figure 1). The epithelium of some interlobular bile ducts was focally necrotic with neutrophil infiltration, but bile duct loss could not be demonstrated Around these portal tracts a severe interface inflammation was seen with hydropic and rosetting juxtaportal hepatocytes surrounded by a lymphoplasmacytic infiltrate. The patient started treatment with azathioprine 1.5 mg/ kg daily while the dosage of prednisolone was slowly reduced to 7.5 mg every second day This resulted in a normalization of ALAT and ESR while IgG was still slightly elevated around 18 g/L. In order to rule out progressive biliary damage an MRCP was done, which was still normal
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