Autoimmune hepatitis with superimposition of primary sclerosing cholangitis on non-specific chronic ulcerative colitis

Introduction and Objetives

Chronic nonspecific ulcerative colitis can be associated with autoimmune hepatitis (AH) and Primary Sclerosing Cholangitis (PSC) as an overlap (prevalence 1.7-12.6%). The evolution depends on the clinical picture, biochemical pattern, and histological determination. The response to immunosuppressive treatment of inflammatory bowel disease and autoimmune hepatitis is good, but not Primary Sclerosing Cholangitis.

Case Summary

26-year-old female resident of Mexico City. Family history of arterial hypertension and acute myocardial infarction. Smoking suspended, alcoholism denied, other history denied. Current condition: Begins 2013 with diarrhea with scant blood, bloating, abdominal pain, general malaise, weight loss; microcytic hypochromic anemia, thrombocytosis, hypertransaminasemia. Colonoscopy: Pancolitis Mayo 2. Biopsy: chronic ulcerative colitis, intense cryptitis, cryptic abscesses. Hypertransaminasemia and cholestatic syndrome persisted; viral hepatitis was ruled out, positive ANAP 1:80, and negative anti-smooth muscle. Liver biopsy: lymphoplasmacytic infiltrate without cholangiole damage or cholestasis, suggestive of HA. He received steroid, azathioprine, ursodeoxycholic acid and mesalazine. Treated latent TB. He received infliximab with improvement. August 2020: jaundice and direct hyperbilirubinemia. Cholangioresonance 2021: Primary Sclerosing Cholangitis. Liver biopsy 2021: lymphocytic infiltrate beyond the limiting plate, plasma cells, cholangiole proliferation, peripheral sclerosis, intracytoplasmic and canalicular cholestasis, focal necrosis, bridges of fibrosis (F3) compatible with AH and PBC. Biological therapy was suspended, Azathioprine was adjusted, and sent for transplant by MELD of 20. Fig. 1 y Fig. 2.

Results:

Conclusions

The patient debuted with moderate Montreal E3S2 UC and confirmed HA. She presented biochemical and clinical remission and intermittent cholestasis; She presented jaundice six years later, confirming PSC by MRI and Biopsy. The evolution of this overlap depends on age, gender and initial phenotype, regardless of the course of UC, representing a diagnostic and therapeutic challenge

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.

Figure 1. Enzyme evolution

Fig. 2.