The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis.
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.
Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy. Electronic address: annarosa.floreani@unipd.it.
Annarosa
A
Floreani
Department of Medicine, University of Padova, Italy.
Raffaella
R
Motta
Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy.
Nora
N
Cazzagon
Department of Surgery, Oncology and Gastroenterology, University of Padova, Italy.
Irene
I
Franceschet
Pathology Unit, Humanitas Research Hospital & University of Milan Medical School, Italy.
Massimo
M
Roncalli
Department of Medicine, University of Padova, Italy.
Teresa
T
Del Ross
Gastroenterologia & Epatologia, Presidio Sanitario Gradenigo, Torino, Italy.
Floriano
F
Rosina
Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano, MI, Italy.
Ana
A
Lleo
Department of Medicine, University of Padova, Italy.
Claudia
C
Mescoli
Liver Unit, Seriate Hospital, Bergamo, Italy.
Guido
G
Colloredo
Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano, MI, Italy.
Pietro
P
Invernizzi
D015535:Arthritis, Psoriatic / Q000097:blood / Q000175:diagnosis / Q000473:pathology; D001323:Autoantibodies / Q000097:blood; D015209:Cholangitis, Sclerosing / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D015897:Comorbidity; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology*; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D013577:Syndrome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D001323:Autoantibodies; D014409:Tumor Necrosis Factor-alpha; D014580:Ursodeoxycholic Acid
Overlap syndrome; Primary biliary cirrhosis; Primary sclerosing cholangitis