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New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome.

ABSTRACT: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune-mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histologic features of AIH and PBC along with modifications of biochemical and immunologic characteristics. We evaluated 272 patients with chronic liver disease, including 105 with AIH, 102 with PBC, and 65 with OS. The best performance for the diagnosis of OS was noted among patients with an overlap score of ?21 who had a sensitivity of 98.5%, a specificity of 92.8%, a positive predictive value of 81.0%, and a negative predictive value of 99.5%. By using a cut-off score of 21, 64 (98.5%) patients were diagnosed with OS as opposed to 9 (8.8%) and 6 (5.7%) with PBC and AIH, respectively. All patients with OS had an aggregate score of >19, whereas most patients with PBC or AIH scored <19, making this a safe discriminatory cut-off point against OS. Conclusion: The new scoring system for the diagnosis of OS has a high sensitivity and specificity for scores ?21, while a score <19 suggests a diagnosis other than OS. This classification can identify patients and diagnose OS with a reasonable amount of accuracy and may be superior to current OS scoring systems in detecting mild forms of OS. (Hepatology Communications 2018;2:245-253).

SUBMITTER: Zhang W

PROVIDER: S-EPMC5831022 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature

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New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome.

Zhang Wei W De Debapriya D Mohammed Kahee A KA Munigala Satish S Chen Guilan G Lai Jin-Ping JP Bacon Bruce R BR

Hepatology communications 20180220 3

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two major immune-mediated chronic liver diseases. Overlap syndrome (OS) is diagnosed if patients have features of both AIH and PBC; however, there is no consensus on the definition or diagnostic criteria for OS. Here, we report a new scoring classification for OS and evaluate its usefulness. This new scoring classification was developed by modifying the International Autoimmune Hepatitis Group classification by selecting histol ...[more]

PMID: 29507900

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Project description:BackgroundA 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.AimTo 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.MethodsIn 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.ResultsThe 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%.ConclusionPSC and autoimmune hepatitis overlap syndrome is generally associated with inflammatory bowel disease and has low mortality and good response to treatment.

Dataset Information

New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome.

Publications

New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome.

Similar Datasets

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