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Antineutrophil cytoplasmic antibody profiles differ according to type of primary sclerosing cholangitis and autoimmune hepatitis

  • Crescente, Juliana Goldbaum1, 2
  • Dellavance, Alessandra3, 4
  • Diniz, Marcio Augusto5
  • Carrilho, Flair Jose1
  • de Andrade, Luis Eduardo Coelho3, 4
  • Cançado, Eduardo Luiz Rachid1, 2
  • 1 Departamento de Gastroenterologia, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, BR
  • 2 Laboratorio de Investigacao Medica (LIM 06), Instituto de Medicina Tropical, Universidade de Sao Paulo, Sao Paulo, SP, BR
  • 3 Disciplina de Reumatologia, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo, SP, BR
  • 4 Divisao de Pesquisa e Desenvolvimento, Laboratorios Fleury Medicina e Saude, Sao Paulo, SP, BR
  • 5 Biostatistics and Bioinformatics Research Center, Samuel Oschin Comprehensive Cancer Institute, Cedars Sinai Medical Center, Los Angeles, CA, USA
Published Article
Faculdade de Medicina / USP
Publication Date
Feb 01, 2021
DOI: 10.6061/clinics/2021/e2228
PMID: 33567045
PMCID: PMC7847259
PubMed Central


OBJECTIVES: To determine the frequency of the antineutrophil cytoplasmic antibodies (ANCA), antiproteinase-3 and antimyeloperoxidase, in primary sclerosing cholangitis (PSC) with or without inflammatory bowel disease (IBD+ or IBD-) and in different types of autoimmune hepatitis (AIH). Additionally, to verify the agreement between ANCA patterns by indirect immunofluorescence and their antigenic specificities by ELISA. METHODS: For this study, 249 patients were enrolled (42 PSC/IBD+; 33 PSC/IBD-; 31 AIH type-1; 30 AIH type-2; 31 AIH type-3; 52 primary biliary cirrhosis; 30 healthy controls) whose serum samples were tested for ANCA autoantibodies. RESULTS: There were fewer female subjects in the PSC/IBD- group ( p =0.034). Atypical perinuclear-ANCA was detected more frequently in PSC/IBD+ patients than in PSC/IBD- patients ( p =0.005), and was significantly more frequent in type-1 ( p <0.001) and type-3 AIH ( p =0.012) than in type-2 AIH. Proteinase-3-ANCA was detected in 25 samples (only one with cytoplasmic-ANCA pattern), and more frequently in PSC/IBD+ than in PSC/IBD- patients ( p =0.025). Myeloperoxidase-ANCA was identified in eight samples (none with the perinuclear-ANCA pattern). Among the 62 reactive samples for atypical perinuclear-ANCA, 13 had antigenic specific reactions for proteinase-3 and myeloperoxidase. CONCLUSIONS: PSC/IBD+ differed from PSC/IBD- in terms of sex and proteinase 3-ANCA and atypical perinuclear-ANCA reactivity, the latter of which was more frequently detected in type-1 and type-3 AIH than in type-2 AIH. There was no agreement between ANCA patterns and antigenic specificities in IBD and autoimmune liver diseases, which reinforces the need for proteinase-3 and myeloperoxidase antibody testing.

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