Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

Abstract

The development of sclerosing cholangitis (SC) is observed in up to 50% of children followed up for autoimmune hepatitis (AIH). In adults, the prevalence is less known, although a recent study found evidence of SC in 10% of AIH patients using magnetic resonance cholangiopancreatography (MRCP). The aim of this study was to assess prospectively the prevalence of SC in adults with AIH. Fifty-nine consecutive patients with AIH diagnosed according to International Autoimmune Hepatitis Group score (women, 71%; mean age, 48 years; cirrhosis, 23%) underwent both MRCP and percutaneous liver biopsy. Twenty-seven patients with cirrhosis of nonbiliary or non-autoimmune etiology served as controls. Fourteen AIH patients (24%) showed mild MRCP abnormalities of intrahepatic bile ducts (IHBDs). None had abnormal common bile duct or convincing evidence of SC on MRCP or biopsy. A diagnosis of overlapping SC was nevertheless retained in one patient with MRCP abnormalities who subsequently developed symptomatic cholestasis despite corticosteroid therapy. Fibrosis score was the only independent parameter associated with bile duct abnormalities on MRCP (odds ratio 2.4; 95% confidence interval 1.4-4.7) and the percentage of patients with IHBD MRCP abnormalities was not different among F3-F4 AIH patients (n ‫؍‬ 24) and cirrhotic controls (46% versus 59%; NS). Conclusion: In this cohort of adult patients with AIH, the prevalence of SC was much lower than previously reported (1.7%). Mild MRCP abnormalities of IHBD were seen in a quarter of patients, but these abnormalities resulted from hepatic fibrosis and not SC. In the absence of cholestatic presentation, MRCP screening does not seem justified in adult-onset AIH. (HEPATOLOGY 2009;50:528-537.)

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

“…Using the same strategy, other authors have reported a frequency of AIH-PSC overlap in 7,4%-14% of the patients with PSC using the revised AIHSG criteria (176) . On the other hand, cholangiographic abnormalities, typical of PSC, were reported in 49% of the children with AIH type 1 and only in 1,7%-10% of the adults with the disease (8,179,180) . It should be pointed out that the diagnosis of overlap syndromes of ALD is not straightforward.…”

Comparative study of liver biopsy results in egypt over three decades

“…b) Primary Sclerosing Cholangitis (PSC) that can also be an indication for liver biopsy, however other non-invasive techniques can be diagnostic [30].…”

Overlap Syndromes

“…When the revised IAIHG criteria were applied to a large series of PSC patients, the prevalence of PSC-AIH overlapping features ranged from 7 to 14% [12,32,33] . On the other hand, cholangiographic abnormalities suggesting PSC are found in AIH patients at a various prevalence depending on the age of patients evaluated: 2-10% in adults [34,35] (41% if ulcerative colitis is present [36] ), and up to 50% in children [37] . The diagnosis of large duct PSC should always be established on typical cholangiographic findings, keeping in mind that an intrahepatic biliary tree, which simulates a sclerosing pattern can be observed in any liver disease with extensive fibrosis [34] .…”

“…On the other hand, cholangiographic abnormalities suggesting PSC are found in AIH patients at a various prevalence depending on the age of patients evaluated: 2-10% in adults [34,35] (41% if ulcerative colitis is present [36] ), and up to 50% in children [37] . The diagnosis of large duct PSC should always be established on typical cholangiographic findings, keeping in mind that an intrahepatic biliary tree, which simulates a sclerosing pattern can be observed in any liver disease with extensive fibrosis [34] . Some cases of small duct PSC (normal cholangiogram)-AIH OS have also been reported, but it can be argued that approximately 10% of patients with typical AIH, with or without ulcerative colitis, may have histological features of bile duct injury [38] .…”

“…These findings also suggest the need of an investigation of the biliary tree at least with MRCP in all children with a diagnosis of AIH [39] . At present, this variant seems unique for children, as a prospective study in adults with AIH was negative [34] and thus, in the absence of cholestatic indices, MRCP screening does not seem justified in adult-onset AIH. However, particularly in young adults with AIH and cholestatic features or IBD, and in AIH patients with remaining cholestasis despite adequate immunosuppression, MRCP is recommended for the detection of possible underlying or co-existent PSC.…”