Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis

Abstract

Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is a clinical entity characterized by the occurence of both conditions at the same time in the same patient. In addition to PBC-AIH overlap syndrome, transitions from one autoimmune disease to another have been reported, but no systematic series have been published. We report a series of 12 patients with consecutive occurrence of PBC and AIH (i.e., PBC followed by AIH). Among 282 PBC patients, 39 were identified who fulfilled criteria for probable or definitive AIH. AIH developed in 12 patients (4.3%). The baseline characteristics of the patients were similar to those of patients with classical PBC. Time elapsed between the diagnosis of PBC and the diagnosis of AIH varied from 6 months to 13 years. Patients with multiple flares of hepatitis at the time of diagnosis of AIH had cirrhosis on liver biopsy. Ten patients were given prednisone ؎ azathioprine; short-term as well as sustained remissions were obtained in 8 of these, while two had multiple relapses and eventually died 8 and 7 years after diagnosis of AIH. In conclusion, the development of superimposed AIH could not be predicted from baseline characteristics and initial response to UDCA therapy. If not detected early, superimposed AIH can result in rapid progression toward cirrhosis and liver failure in PBC patients. (HEPATOLOGY 2006;44:85-90.)

The Development of Autoimmune Hepatitis and Primary Biliary Cirrhosis Overlap Syndrome During the Course of Connective Tissue Diseases: Report of Three Cases and Review of the Literature

“…The time interval between the diagnosis of PBC and the diagnosis of AIH varied from 6 months to 13 years. Of importance, patients with multiple flares of hepatitis at the time of diagnosis of AIH had already developed cirrhosis on liver biopsy [12]. In the literature, the development of AIH or PBC separately has been reported with CTD in several cases, but we could not find any AIH-PBC overlap with CTD.…”

“…AIH-PBC overlap syndrome is the most common form of hepatic overlap syndrome, affecting almost 10% of patients with AIH or PBC [2,12]. In addition to concomitant AIH-PBC cases, sequential overlap may also occur (PBC to AIH or AIH to PBC).…”

Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

“…This categorization is of more than academic importance because it has implications for outcome and treatment. There is now good evidence that patients with PBC associated with florid interface hepatitis respond to immunosuppressive therapy whereas those in whom the biliary features predominate do not and should be treated with ursodeoxycholic acid alone [42,46,47]. Why hepatitis should be more steroid sensitive than biliary inflammation is not clear.…”

Overlap Syndromes

“…In most cases, the 'dominant' disease phenotype is PBC [12] . Less commonly, the onset of AIH and PBC is temporally dissociated, usually with PBC presenting first with a variable interval of 6 months to 13 years before the onset of AIH [22] . The development of AIH could not be predicted from baseline characteristics and initial response to UDCA.…”

“…Interestingly, it has been suggested that, in responders, doses of immunosuppressants (IS) in the long term could be lower and the rate of successful withdrawal higher than in classical AIH [24,28] . In UDCA-treated PBC developing AIH ('sequential' OS), the use of immunosuppressive treatment is mandatory [22,29] .…”