Annarosa Floreani1, Raffaella Motta2, Nora Cazzagon3, Irene Franceschet3, Massimo Roncalli4, Teresa Del Ross2, Floriano Rosina5, Ana Lleo6, Claudia Mescoli2, Guido Colloredo7, Pietro Invernizzi6. 1. Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy. Electronic address: annarosa.floreani@unipd.it. 2. Department of Medicine, University of Padova, Italy. 3. Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy. 4. Pathology Unit, Humanitas Research Hospital & University of Milan Medical School, Italy. 5. Gastroenterologia & Epatologia, Presidio Sanitario Gradenigo, Torino, Italy. 6. Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano, MI, Italy. 7. Liver Unit, Seriate Hospital, Bergamo, Italy.
Abstract
BACKGROUND: The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis is an extremely rare condition that has been reported in only six published cases so far. METHODS: Here we report two cases showing the clinical manifestations of both primary biliary cirrhosis and primary sclerosing cholangitis. RESULTS: In one case the overlap condition was associated with psoriatric arthritis, and the patient successfully underwent dual treatment with ursodeoxycholic acid and the anti-tumour necrosis factor-alpha agent adalimumab. In the second case, the predominant condition was, initially, an antimitochondrial antibody-negative primary biliary cirrhosis with progressive course towards end-stage liver disease; the patient then developed either antimitochondrial antibody positivity or changes in the biliary tree compatible with primary sclerosing cholangitis. CONCLUSIONS: These two cases add information on a controversial issue in the literature, and indicate the importance of recognizing a possible overlap syndrome to optimize treatment.
BACKGROUND: The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis is an extremely rare condition that has been reported in only six published cases so far. METHODS: Here we report two cases showing the clinical manifestations of both primary biliary cirrhosis and primary sclerosing cholangitis. RESULTS: In one case the overlap condition was associated with psoriatric arthritis, and the patient successfully underwent dual treatment with ursodeoxycholic acid and the anti-tumour necrosis factor-alpha agent adalimumab. In the second case, the predominant condition was, initially, an antimitochondrial antibody-negative primary biliary cirrhosis with progressive course towards end-stage liver disease; the patient then developed either antimitochondrial antibody positivity or changes in the biliary tree compatible with primary sclerosing cholangitis. CONCLUSIONS: These two cases add information on a controversial issue in the literature, and indicate the importance of recognizing a possible overlap syndrome to optimize treatment.
Authors: Marco Fiore; Sebastiano Leone; Alberto Enrico Maraolo; Emilio Berti; Giovanni Damiani Journal: Biomed Res Int Date: 2018-02-06 Impact factor: 3.411