Abstract
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.
Highlights
Antineutrophil cytoplasmic antibodies with atypical perinuclear patterns (A-antineutrophil cytoplasmic antibodies (ANCA)) are frequently found in inflammatory bowel diseases (IBD), in ulcerative colitis, primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) [1,2]
The most significant difference was the higher prevalence of women in the PSC/IBD- group than in the PSC/IBD+ group (57.6% vs. 31.0%, respectively, p=0.034)
There was no significant difference in the frequency of A-ANCA between PSC/IBD- and primary biliary cholangitis (PBC) (p= 0.56)
Summary
Antineutrophil cytoplasmic antibodies with atypical perinuclear patterns (A-ANCA) are frequently found in inflammatory bowel diseases (IBD), in ulcerative colitis, primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) [1,2]. Since PSC and IBD are closely associated, it is not clear whether A-ANCA reactivity is more. No potential conflict of interest was reported. Received for publication on July 21, 2020. Accepted for publication on December 9, 2020. Few studies have addressed the frequency distribution of this marker in IBD-associated (PSC/IBD+) and un-associated PSC (PSC/IBD-)
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