Overlay of autoimmune cholangitis and autoimmune hepatitis

Introduction and Objectives

The "overlap syndrome" is a variant of autoimmune hepatitis (AIH) in addition to cholestatic liver disease. AIH can present concurrently with primary biliary cholangitis (PBC) 7% to 13%, primary sclerosing cholangitis (PSC) 6% to 11% or autoimmune cholangitis (AIC) 3% to 9%, the rarest and associated with a poor prognosis. Diagnosis requires biochemical alteration, immunological studies and biopsy, plus the exclusion of viral, toxic, metabolic and hereditary etiologies. Therapy, including corticosteroids, ursodeoxycholic acid and immunosuppressants, should be individualized and guided by the severity of the cholestasis findings.

Materials and Methods

66-year-old female. She presented in 2018 with the detection of hepatic steatosis, a weight loss of 32 kg in 2 years. Asthenia, adynamia, pruritus and scleral jaundice progressing to generalized. In laboratories: BT 11.37 (BI 8.5), AST 187, ALT 148, GGT 1761, FA 1819, ANAs positive anti centromere 1:40 and Hep-2 cells 1:640, cholangioresonance without data of CEP. Treatment with ursodeoxycholic acid was started, with no response, and a liver biopsy was performed compatible with HAI+CAI, Fig. 1 and 2. We started therapy with prednisone and azathioprine.

Conclusions

Recognizing that AIH and IAC are diseases with high morbidity that progress to chronic liver damage with fibrosis and cirrhosis, their early identification would help in the establishment of timely and effective treatment.

Funding

The resources used in this study were from the hospital without any additional financing

Declaration of interest

The authors declare no potential conflicts of interest.

Fig.1: Masson's trichrome stain: fibrous portal expansion with the formation of incomplete portal-port bridges.

Fig. 2: No bile duct, granulomatous inflammation with inflammatory infiltrate predominantly lymphocytes, plasma cells and epithelioid macrophages that exceed the limiting plaque. Intracellular cholestasis.