Innumerable Liver Masses in a Patient with Autoimmune Hepatitis and Primary Sclerosing Cholangitis Overlap Syndrome.
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.
Gastrointestinal and Liver Diseases Research Center (GLDRC), Guilan University of Medical Sciences, Rasht, Iran.
Alireza
A
Gharibpoor
Gastrointestinal and Liver Diseases Research Center (GLDRC), Guilan University of Medical Sciences, Rasht, Iran.
Fariborz
F
Mansour-Ghanaei
Gastrointestinal and Liver Diseases Research Center (GLDRC), Guilan University of Medical Sciences, Rasht, Iran.
Mahbobe
M
Sadeghi
Gastrointestinal and Liver Diseases Research Center (GLDRC), Guilan University of Medical Sciences, Rasht, Iran.
Faeze
F
Gharibpoor
Gastrointestinal and Liver Diseases Research Center (GLDRC), Guilan University of Medical Sciences, Rasht, Iran.
Farahnaz
F
Joukar
Guilan University of Medical Sciences (GUMS), Gastrointestinal and Liver Diseases Research Center (GLDRC), Rasht, Iran.
Sara
S
Mavaddati
D000328:Adult; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D003937:Diagnosis, Differential; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000473:pathology; D008297:Male; D012307:Risk Factors; D013577:Syndrome; D016896:Treatment Outcome
D007166:Immunosuppressive Agents