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IDENTICAL TWIN BROTHERS WITH UC AND DISCORDANT HEPATOBILIARY MANIFESTATIONS OF IBD
A Martinez, R Schreiber, D Israel, K Jacobson
Division of Pediatric Gastroenterology and Nutrition, Department of Pediatrics, BC Children’s Hospital, Vancouver, British Columbia
INTRODUCTION: The etiology of IBD involves genetic factors. While concordance rates of Crohn’s or UC in identical twins are well described, little is known of the hepatobiliary manifestations of IBD in these cases. Herein we report identical twins having UC with discordance for immune liver disease.
METHODS: The hospital records of twin brothers with UC were reviewed. Clinical, biochemical and histological findings were recorded. A literature review was performed.
RESULTS: Twin A presented at age 4 yrs with bloody diarrhea and weight loss. Colonoscopy found distal disease and the histological findings were consistent with UC. At presentation the AST 160U/L; ALT 216U/L; GGT 240U/L; ALP 143U/L; IgG 21 g/L, ANA 1:160; and ASMA 1:40. Liver biopsy showed AIH: chronic interface hepatitis with normal bile ducts. He received induction therapy with Mesalamine and Prednisone. 6MP was introduced and complete remission has been maintained off steroids. Twin B presented at age 11 yrs with bloody diarrhea and normal liver biochemistries. Colonoscopy revealed pancolitis; the histological findings confirmed UC. Mesalamine induced remission. One year later he developed fatigue and RUQ pain. AST 46U/L, ALT 80U/L, GGT 110U/L, ALP 119U/L, ANA negative. Liver biopsy showed PSC: increased collagen deposition around bile ducts without hepatitis. Ursodiol led to normalization of the liver enzymes. A literature review found few reports of PSC, but not AIH, occurring in twins or siblings with UC.
CONCLUSIONS: To our knowledge this is the first report of discordant hepatobiliary disease in twins with UC, with one brother having AIH and the other having PSC. While genetics play an important role in disease pathogenesis, it is likely that environmental modifiers are also involved.