@misc{LopensKrawczykPappetal., author = {Lopens, Steffi and Krawczyk, Marcin and Papp, Maria and Milkiewicz, Piotr and Schierack, Peter and Liu, Yudong and Wunsch, Ewa and Conrad, Karsten and Roggenbuck, Dirk}, title = {The search for the Holy Grail: autoantigenic targets in primary sclerosing cholangitis asscociated with diesease phenotype an neoplasia}, series = {Autoimmunity Highlights}, volume = {11}, journal = {Autoimmunity Highlights}, issn = {2038-3274}, doi = {10.1186/s13317-020-00129-x}, pages = {14}, language = {en} } @misc{LopensWunschMilkiewiczetal., author = {Lopens, Steffi and Wunsch, Ewa and Milkiewicz, Malgorzata and R{\"o}ber, Nadja and Zarske, Grit and Nasser, Abdullah and Conrad, Karsten and Laass, Martin W. and R{\"o}diger, Stefan and Krawczyk, Marcin and Roggenbuck, Dirk and Milkiewicz, Piotr}, title = {PR3-ANCAs Detected by Third-Generation ELISA Predicts Severe Disease and Poor Survival in Primary Sclerosing Cholangitis}, series = {Diagnostics}, volume = {12}, journal = {Diagnostics}, number = {11}, issn = {2075-4418}, doi = {10.3390/diagnostics12112682}, abstract = {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.}, language = {en} }