The coexistence of primary biliary cirrhosis and autoimmune hepatitis has been called 'overlap syndrome'.
However, diagnosis is challenging and the natural history of this syndrome has not been demonstrated.
The importance of the diagnosis of overlap is due to potential therapeutic options.
Patients with primary biliary cirrhosis should receive ursodeoxycholic acid.
The role of and response to additional immunosuppressive therapy are unknown when autoimmune hepatitis overlaps primary biliary cirrhosis.
|There was a higher rate of death in the overlap group|
|The American Journal of Gastroenterology|
Dr Marina Silveira and colleagues from Minnesota, USA reviewed 135 patients with primary biliary cirrhosis.
The research team used a revised scoring system proposed by the International Autoimmune Hepatitis Group.
The team noted that 26 patients had features of primary biliary cirrhosis-autoimmune hepatitis overlap, and 109 did not.
The mean follow-up was 6 years for overlap syndrome patients and 5 years in primary biliary cirrhosis patients.
The team found a higher rate of portal hypertension, esophageal varices, and gastrointestinal (GI) bleeding in the overlap group.
Ascites, and death and/or orthotopic liver transplantation in the overlap group.
Esophageal varices, GI bleeding, ascites, and death and/or orthotopic liver transplantation were more common in the overlap group.
Dr Silveira's team concluded, "The higher risk of symptomatic portal hypertension and worse outcomes in patients with primary biliary cirrhosis overlap syndrome may justify the risks of immunosuppressive therapy."
"Large randomized studies are necessary to establish optimal therapeutic strategies."