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.

Non-alcoholic fatty liver disease has become the leading chronic liver disease in the developed world, with a prevalence of 6%-35%. Its pathological spectrum ranges from simple steatosis (non-alcoholic fatty liver) to different degrees of inflammation and liver cell damage [non-alcoholic steatohepatitis (NASH)]. NASH has gained attention in recent years because of its association with hepatocellular carcinoma (HCC). Although the occurrence of HCC is more frequent in the presence of cirrhosis, studies have shown that hepatic carcinogenesis may also develop in the context of NASH without association with advanced fibrosis, as well as from simple steatosis. Evidence of the onset of HCC in the absence of cirrhosis is of concern, since recent surveillance and screening guidelines for liver cancer do not include this population subgroup. Therefore, it is imperative that new effective screening and monitoring measures for HCC, or even the reformulation of these recommendations, be taken to handle these patients considered to be at high risk. The present paper aims to review the literature on the occurrence of HCC in patients with NASH with or without cirrhosis. In addition, we report a case showing the development of HCC in a patients with NASH without cirrhosis.

Female patient, 61 years-old, heavy smoker, and heavy drinker, sought care because of watery diarrhea, severe weight loss (around 40 pounds), alteration of mental state, and skin rash. She presented with signs of dementia (blurry speech, mental confusion, and amnesia) and severe malnutrition. Initial investigation had shown anemia (hemoglobin = 8.5 g/dL), low albumin (2.05 g/dL), and an increase of liver enzymes (GGT = 450 U/L and alkaline phosphatase = 287 U/L). Serological tests for syphilis, HIV, hepatitis C virus, and hepatitis B virus were negative. Parasitological stool examination and fecal leukocyte tests were normal, anti-transglutaminase IgA and IgG antibodies were negative, and she had a normal oral lactose tolerance test. Colonoscopy was performed, showing normal mucosa with a normal anatomopathological study of colon biopsies. The skin rash, tough, was very relevant for the case: hyperpigmentation, scaling, and skin thickening on the face, upper chest, and upper and lower limbs-areas exposed to sunlight (Figs. 1, 2). A presumptive diagnosis of pellagra was made by the clinical gastroenterology team. Supplementation of nicotinamide in the dose of 50 mg QID was began and maintained for 28 days, with resolution of diarrhea and of the skin rash, a substantial weight gain, and a considerable improvement of mental status. Because in Brazil there is no commercial nicotinamide presentation, it was used 20 daily tablets of complex B vitamins, which contain 10 mg of nicotinamide each. The patient was discharged with maintenance supplementation of complex B vitamins and a plan for treatment of alcoholism. Pellagra is a disease caused by the deficiency of niacin (vitamin B3), first described in 1735 by the Spanish physician Gaspar