Patients with autoimmune liver disease in a frequency of 7% -18% fit into the diagnostic criteria for more than one condition. Up to 12.5% of Autoimmune Hepatitis (AIH) and Primary Sclerosing Cholangitis (PSC) cohorts have a label of AIH/PSC overlap. There can be an unpredictable interval of many years between the diagnosis of the two conditions. There are scoring systems but no established criteria for the diagnosis of AIH/PSC overlap. Therefore, a diagnosis based on combination of biochemistry, autoantibody profile, cholangiogram, and liver histologyis made. Histopathology staging of AIH/PSC overlap influences therapeutic options and prognosis. There is beneficial role of immunosuppression, even though there is a higher relapse rate and evidence of progressive liver disease despite immunosuppression in some cases. Liver related outcomes in this overlap are better than PSC alone but are poorer than AIH. We herein report a rare presentation of an overlap syndrome with AIH and PSC in a patient in whom, treatment with first line therapy showed incomplete response and the salvage therapy was not tolerated, presenting with Acute Kidney Injury (AKI) and a clinical picture of sepsis resembling AIH flare.
Applicability and prognostic value of histologic scoring systems in primary sclerosing cholangitis