TY - GEN A1 - Lopens, Steffi A1 - Wunsch, Ewa A1 - Milkiewicz, Malgorzata A1 - Röber, Nadja A1 - Zarske, Grit A1 - Nasser, Abdullah A1 - Conrad, Karsten A1 - Laass, Martin W. A1 - Rödiger, Stefan A1 - Krawczyk, Marcin A1 - Roggenbuck, Dirk A1 - Milkiewicz, Piotr T1 - PR3-ANCAs Detected by Third-Generation ELISA Predicts Severe Disease and Poor Survival in Primary Sclerosing Cholangitis T2 - Diagnostics N2 - A highly sensitive detection of anti-neutrophil cytoplasmic antibodies to serine proteinase-3 (PR3-ANCAs) aids in the serological diagnosis of autoimmune liver disorders and the prediction of severity in primary sclerosing cholangitis (PSC). Here, we evaluate a novel third-generation ELISA for the detection of PR3-ANCAs. In total, 309 patients with PSC, 51 with primary biliary cholangitis (PBC), and 120 healthy blood donors (BD) were analyzed. For the survival analysis in PSC, the outcome was defined as liver-transplantation-free survival during the follow-up. Positive PR3-ANCA levels were found in 74/309 (24.0%) of patients with PSC. No BDs and one patient with PBC demonstrated PR3-ANCA positivity. PR3-ANCAs were revealed as independent predictors for a poor PSC outcome (study endpoint: liver transplantation/death, log-rank test, p = 0.02). PR3-ANCA positivity, lower albumin levels, and higher bilirubin concentrations were independent risks of a poor survival (Cox proportional-hazards regression analysis, p < 0.05). The Mayo risk score for PSC was associated with PR3-ANCA positivity (p = 0.01) and the disease severity assessed with a model of end-stage liver disease (MELD) and extended MELD-Na (p < 0.05). PR3-ANCAs detected by a third-generation ELISA are diagnostic and prognostic markers for PSC. Their wider use could help to identify patients who are at-risk of a more severe disease. KW - primary sclerosing cholangitis KW - antibodies against serine protease proteinase-3 KW - survival KW - health-related quality of life KW - liver biochemistry KW - disease severity Y1 - 2022 UR - https://opus4.kobv.de/opus4-UBICO/frontdoor/index/index/docId/29710 SN - 2075-4418 VL - 12 IS - 11 ER -