Evolution of autoimmune hepatitis to primary sclerosing cholangitis: A sequential syndrome

Abstract

Recently, the autoimmune hepatitis (AIH)/primary sclerosing cholangitis (PSC) overlap syndrome has been reported increasingly. In this syndrome, patients present with features of both AIH and PSC. It has been suggested that the 2 diseases may be sequential in their occurrence, whereby patients have features of AIH and then after a number of years develop features of PSC, but clear confirmation of evolution has not been documented in adults. We describe 6 adult cases in which PSC was diagnosed many years after well-established AIH. Six patients are described in whom AIH definitely was diagnosed at presentation. No evidence of biliary disease was noted on the initial liver biopsy or endoscopic retrograde cholangiography (ERCP). All patients responded well to immunosuppressive therapy. After an average duration of follow-up of 4.6 years they became resistant to immunosuppression, and developed clear features of PSC, which was confirmed by ERCP in all patients. The average age of the patients at first presentation was 31.3 years, 2 were women and 4 were men, and 3 had ulcerative colitis. We found no specific features at presentation that could predict this evolutionary outcome. In conclusion, patients with well-established AIH can, after variable duration of follow-up, develop PSC. In patients with AIH who become resistant to immunosuppression or develop significant cholestasis, PSC should be ruled out by ERCP.

Liver transplantation for overlap syndromes of autoimmune liver diseases

“…Interestingly, all patients with overlap syndromes AIH‐PSC had multiple risk factors for recurrence, as prior studies have shown that inflammatory bowel disease , recurrent acute cellular rejection (ACR) and intact colon to be risk factors for PSC recurrence. In this study, patients with overlap syndromes AIH‐PSC had ulcerative colitis and intact colon, and each experienced at least one episode of ACR, which may explain the high frequency of recurrence in this subgroup of patients.…”

“…However, when the revised International Autoimmune Hepatitis Group (IAIHG) criteria are used, between 2 and 19% of patients with PBC have overlap syndromes . Similarly, 7 to 14% of patients with PSC meet the criteria for overlap syndromes when the revised IAIHG criteria are employed , and these patients also tend to have a worse prognosis .…”

British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis

“…The prevalence of AIH appears to be higher in children with PSC 305 306. PSC may develop many years after a diagnosis of AIH 307. The prevalence of large duct PSC in adults with AIH has recently been reported as 2% and 10% based on magnetic resonance cholangiopancreatography (MRCP) surveys 77 78.…”

Liver Disorders in Inflammatory Bowel Disease

“…This disorder is well described in patients with IBD especially UC [39]. There is published data that shows patients with IBD and AIH who subsequently go on to develop PSC [38, 40, 41]. The significance of this relationship with IBD is still unclear, and the onset of disease can occur at anytime before or after the diagnosis of IBD.…”