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001-es BibID:	BIBFORM083946
Első szerző:	Lopens, Steffi
Cím:	The search for the Holy Grail : autoantigenic targets in primary sclerosing cholangitis associated with disease phenotype and neoplasia / Steffi Lopens, Marcin Krawczyk, Maria Papp, Piotr Milkiewicz, Peter Schierack, Yudong Liu, Ewa Wunsch, Karsten Conrad, Dirk Roggenbuck
Dátum:	2020
ISSN:	2038-0305 2038-3274
Megjegyzések:	Unlike in other autoimmune liver diseases such as autoimmune hepatitis and primary biliary cholangitis, the role and nature of autoantigenic targets in primary sclerosing cholangitis (PSC), a progressive chronic immune-mediated, life threatening, genetically predisposed, cholestatic liver illness, is poorly elucidated. Although anti-neutrophil cytoplasmic antibodies (ANCA) have been associated with the occurrence of PSC, their corresponding target(s) have not yet been identified entirely. Genome wide association studies recently revealed a significant number of immune-related and even disease-modifying susceptibility loci for PSC. However, they did not allow to discern a clear autoimmune pattern nor do therapy options and male gender preponderance in PSC support a pathogenic role of autoimmune responses. Yet, there is growing evidence that PSC is closely linked to the co-occurrence of inflammatory bowel disease (IBD) phenotypes. The identification of novel autoantigenic targets interacting with the gut microbiota in IBD such as the major zymogen granule membrane glycoprotein 2 (GP2) or the detection of high-sensitive proteinase 3 (PR3) autoantibodies have refocused the interest on a putative association of loss of tolerance with the IBD phenotype. Not surprisingly, the report of GP2 IgA to be associated with fibrosis in PSC and disease severity gave new impetus to autoantibody research for autoimmune liver illnesses and might usher in a new era of serological research in this field. The mucosal loss of tolerance against the microbiota-sensing GP2 modulating innate and adaptive intestinal immunity and its putative role in the pathogenesis of PSC will be discussed along with other potential PSC-related autoantigenic targets such as the neutrophil PR3 in this review. GP2 IgA may represent a group of new pathogenic antibodies which share characteristics of both type 2 and 3 of antibody-mediated hypersensitive reactions according to Coombs and Gell.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk primary sclerosing cholangitis microbiota ulcerative colitis Crohn's disease inflammatory bowel disease cirrhosis cholangiocarcinoma immunoglobulin A
Megjelenés:	Autoimmunity Highlights. - 11 : 1 (2020), p. 1-14. -
További szerzők:	Krawczyk, Marcin Papp Mária (1975-) (belgyógyász, gasztroenterológus) Milkiewicz, Piotr Schierack, Peter Liu, Yudong Wunsch, Ewa Conrad, Karsten Roggenbuck, Dirk
Internet cím:	Intézményi repozitóriumban (DEA) tárolt változat
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