Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review

Abstract

BACKGROUND A previously healthy 22-year-old woman presented with abdominal pain and jaundice. She had a reagent antinuclear factor (1:640, with a homogeneous nuclear pattern) and hypergammaglobulinemia (2.16 g/dL). Anti-smooth muscle, anti-mitochondrial and anti-liver-kidney microsomal antibody type 1 antibodies were negative. Magnetic resonance cholangiography showed a cirrhotic liver with multiple focal areas of strictures of the intrahepatic bile ducts, with associated dilations. Liver biopsy demonstrated periportal necroinflammatory activity, plasmocyte infiltration and advanced fibrosis. Colonoscopy showed ulcerative pancolitis and mild activity (Mayo score 1), with a spared rectum. Treatment with corticosteroids, azathioprine, ursodeoxycholic acid and mesalamine was initiated, with improvement in laboratory tests. The patient was referred for a liver transplantation evaluation. AIM To report the case of a female patient with autoimmune hepatitis and primary sclerosing cholangitis (PSC) overlap syndrome associated with ulcerative colitis and to systematically review the available cases of autoimmune hepatitis and PSC overlap syndrome. METHODS In accordance with preferred reporting items for systematic reviews and meta-analysis protocols guidelines, retrieval of studies was based on medical subject headings and health sciences descriptors, which were combined using Boolean operators. Searches were run on the electronic databases Scopus, Web of Science, MEDLINE (PubMed), Biblioteca Regional de Medicina, Latin American and Caribbean Health Sciences Literature, Cochrane Library for Systematic Reviews and Opengray.eu. Languages were restricted to English, Spanish and Portuguese. There was no date of publication restrictions. The reference lists of the studies retrieved were searched manually. RESULTS The search strategy retrieved 3349 references. In the final analysis, 44 references were included, with a total of 109 cases reported. The most common clinical finding was jaundice and 43.5% of cases were associated with inflammatory bowel disease. Of these, 27.6% were cases of Crohn’s disease, 68% of ulcerative colitis, and 6.4% of indeterminate colitis. Most patients were treated with steroids. All-cause mortality was 3.7%. CONCLUSION PSC and autoimmune hepatitis overlap syndrome is generally associated with inflammatory bowel disease and has low mortality and good response to treatment.

Liver-side of inflammatory bowel diseases: Hepatobiliary and drug-induced disorders

“…An Italian cohort of PSC/AIH patients showed a lower mean age at presentation and higher alanine aminotransferase (ALT) and aspartate aminotransferase (AST) values compared to “classic” PSC[ 45 ]. There is also a strong association between PSC/AIH and IBD; according to a recent systematic review, IBD was present in 44% of PSC/AIH patients, that was UC in 68% of cases[ 46 ]. Patients with an established diagnosis of AIH who also have IBD should be evaluated for concomitant PSC.…”

Hepatobiliar manifestations in inflammatory bowel disease: A practical approach

“…In IBD, there are no established diagnostic criteria, which makes it even more difficult to establish the real incidence of this entity; on the other hand, its appearance is usually sequential over the years[ 44 , 9 ]. The prevalence of this entity is lower than PSC alone and it is more commonly diagnosed in the pediatric and adolescent population[ 9 , 45 ]. Given the autoimmune nature of the disease associated with cholestasia, the concomitant use of immunosuppressive therapy (corticosteroids and thiopurines) and UDCA is recommended, however, there are no randomized studies that support this strategy.…”

Liver involvement in inflammatory bowel disease: What should the clinician know?

“…A systematic review found approximately 109 cases, which were mostly overlap syndrome with PBC. The authors reported that jaundice was the most common onset sign and that response to steroids was good, with a low mortality rate[ 39 ]. Interestingly, a case report of AIH onset after starting adalimumab has been described, which underlines the possibility that an immunogenic drug may alter an equilibrium in the immune system[ 40 ].…”