07 February 2017 : Case report
Innumerable Liver Masses in a Patient with Autoimmune Hepatitis and Primary Sclerosing Cholangitis Overlap Syndrome
Challenging differential diagnosis, Diagnostic / therapeutic accidents, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis), Rare coexistence of disease or pathology
Alireza Gharibpoor1DE, Fariborz Mansour-Ghanaei1D*, Mahbobe Sadeghi1B, Faeze Gharibpoor1F, Farahnaz Joukar1EF, Sara Mavaddati2EDOI: 10.12659/AJCR.901153
Am J Case Rep 2017; 18:131-135
Abstract
BACKGROUND: In patients with the diagnosis of autoimmune hepatitis (AIH), the presence of cholestatic features raise the possibility of an overlap syndrome with primary sclerosing cholangitis (PSC). Here, we present a unique case with AIH-PSC overlap syndrome and innumerable liver masses.
CASE REPORT: A 26-year-old man presented with generalized icterus. Based on the serological findings of hypergamainmunoglobulinemia and positive anti-nuclear antibody tests, together with an abnormal cholangiogram, he was diagnosed with overlap syndrome (AIH-PSC). Liver imaging revealed innumerable liver masses with a benign appearance in the pathological evaluation. To rule out the colon abnormalities that usually coexist with such liver masses, colonoscopy was performed and showed no significant changes. The liver masses were nonmalignant and were resolved after immunosuppressant therapy.
CONCLUSIONS: Because AIH-PSC overlap syndrome is rare, it is suggested that radiological evaluation of the biliary tree should be performed routinely in adults diagnosed with AIH to reduce the missed diagnosis of overlap syndrome and liver masses.
Keywords: Cholangitis, Sclerosing, Hepatitis, Autoimmune, Liver
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