PMC6346900_01
Family genogram
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His family history was unremarkable except for the presence of PBC in his paternal grandmother (Fig. 1).
Evolution of serum aminotransferases and cholestatic enzymes
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The diagnosis of small duct PSC was proposed, azathioprine and prednisolone were withdrawn and the patient was treated with ursodeoxycholic acid, exhibiting fluctuating levels of cholestasis (Fig. 2). Over the following years, we observed fluctuations in liver enzyme levels (Fig. 2), unrelated to the levels or type of immunosuppression.
Liver histology showing interface and intra-lobular necro-inflammatory activity
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AST and ALT levels and a similar immunological study. A second liver biopsy (Fig. 3) showed portal expansion with polymorphic inflammatory infiltrate with lymphocytic predominance and interface and intra-lobular necro-inflammatory activity.