Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes.
BACKGROUND
The coexistence of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) has been called "overlap syndrome," but diagnosis is challenging and the natural history of this syndrome has not been demonstrated. The importance of the diagnosis of PBC-AIH overlap is due to potential therapeutic options. Patients with PBC should receive ursodeoxycholic acid (UDCA); the role of and response to additional immunosuppressive therapy are unknown when AIH overlaps PBC.


RESULTS
We reviewed 135 patients with PBC according to a revised scoring system proposed by the International Autoimmune Hepatitis Group (IAHG). Twenty-six patients had features of PBC-AIH overlap and 109 did not. Mean follow-up was 6.1 yr for overlap syndrome patients and 5.4 yr in PBC patients. There was a higher rate of portal hypertension (P=0.01), esophageal varices (P<0.01), gastrointestinal (GI) bleeding (P=0.02), ascites (P<0.01), and death and/or orthotopic liver transplantation (OLT) (P<0.05) in the overlap group.


CONCLUSIONS
In conclusion, esophageal varices, GI bleeding, ascites, and death and/or OLT were more common in the overlap group. The higher risk of symptomatic portal hypertension and worse outcomes in patients with PBC overlap syndrome may justify the risks of immunosuppressive therapy. Large randomized studies are necessary to establish optimal therapeutic strategies.
Silveira
Marina G
MG
Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Talwalkar
Jayant A
JA
Angulo
Paul
P
Lindor
Keith D
KD
D001201:Ascites / Q000150:complications; D004932:Esophageal and Gastric Varices / Q000150:complications; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000150:complications; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D014580:Ursodeoxycholic Acid