BACKGROUND: Primary sclerosing cholangitis (PSC) is an immune-mediated biliary disorder of unknown etiology with no effective treatment. The purpose of this study was to better prognosticate the development of cirrhosis, decompensation, and requirement for liver transplantation (LT) in PSC patients based on serum immunoglobulin G4 (IgG4) levels. METHODS: A retrospective chart review was conducted on PSC patients seen at the University of Alberta Hospital between 2002 and 2017. PSC patients were categorized as high IgG4 group (≥70 mg/dL) or normal IgG4 group (<70 mg/dL). Laboratory parameters, clinical characteristics, and outcomes were compared between the groups. RESULTS: One hundred and ten patients were followed over a mean period of 7.3 (SD 5) years. Seventy-two patients (66%) were male, the mean age at diagnosis of PSC was 35 (SD 15) years, and inflammatory bowel disease (IBD) was present in 80 patients (73%). High IgG4 levels were found in 37 patients (34%). PSC patients with high IgG4 had a shorter mean cholangitis-free survival time (5.3 versus 10.4 years, p = 0.02), cirrhosis-free survival time (8.7 versus 13.0 years, p = 0.02), and LT-free survival time (9.3 years versus 18.9 years, p <0.001). IgG4 ≥70 mg/dL was independently associated with liver decompensation and LT-free outcomes. A cut-off IgG4 value of ≥70 mg/dL performed better than a cut-off value of ≥140 mg/dL to predict time to LT (area under the curve [AUC] 0.68, p = 0.03, sensitivity 72%, specificity 78%). CONCLUSIONS: Serum IgG4 ≥70 mg/dL in PSC predicts a shorter time to cirrhosis decompensation and LT.
BACKGROUND: Primary sclerosing cholangitis (PSC) is an immune-mediated biliary disorder of unknown etiology with no effective treatment. The purpose of this study was to better prognosticate the development of cirrhosis, decompensation, and requirement for liver transplantation (LT) in PSC patients based on serum immunoglobulin G4 (IgG4) levels. METHODS: A retrospective chart review was conducted on PSC patients seen at the University of Alberta Hospital between 2002 and 2017. PSC patients were categorized as high IgG4 group (≥70 mg/dL) or normal IgG4 group (<70 mg/dL). Laboratory parameters, clinical characteristics, and outcomes were compared between the groups. RESULTS: One hundred and ten patients were followed over a mean period of 7.3 (SD 5) years. Seventy-two patients (66%) were male, the mean age at diagnosis of PSC was 35 (SD 15) years, and inflammatory bowel disease (IBD) was present in 80 patients (73%). High IgG4 levels were found in 37 patients (34%). PSC patients with high IgG4 had a shorter mean cholangitis-free survival time (5.3 versus 10.4 years, p = 0.02), cirrhosis-free survival time (8.7 versus 13.0 years, p = 0.02), and LT-free survival time (9.3 years versus 18.9 years, p <0.001). IgG4 ≥70 mg/dL was independently associated with liver decompensation and LT-free outcomes. A cut-off IgG4 value of ≥70 mg/dL performed better than a cut-off value of ≥140 mg/dL to predict time to LT (area under the curve [AUC] 0.68, p = 0.03, sensitivity 72%, specificity 78%). CONCLUSIONS: Serum IgG4 ≥70 mg/dL in PSC predicts a shorter time to cirrhosis decompensation and LT.
Authors: Elisabeth M G de Vries; Martti Färkkilä; Piotr Milkiewicz; Johannes R Hov; Bertus Eksteen; Douglas Thorburn; Olivier Chazouillères; Albert Pares; Ståle Nygård; Odd H Gilja; Ewa Wunsch; Pietro Invernizzi; Marco Carbone; Francesca Bernuzzi; Kirsten M Boberg; Helge Røsjø; William Rosenberg; Ulrich H Beuers; Cyriel Y Ponsioen; Tom H Karlsen; Mette Vesterhus Journal: Liver Int Date: 2017-04-13 Impact factor: 5.828
Authors: Lizhi Zhang; Jason T Lewis; Susan C Abraham; Thomas C Smyrk; Stanley Leung; Suresh T Chari; John J Poterucha; Charles B Rosen; Christine M Lohse; Jerry A Katzmann; Tsung-Teh Wu Journal: Am J Surg Pathol Date: 2010-01 Impact factor: 6.394
Authors: Einar Björnsson; Suresh Chari; Marina Silveira; Andrea Gossard; Naoki Takahashi; Thomas Smyrk; Keith Lindor Journal: Am J Ther Date: 2011-05 Impact factor: 2.688
Authors: Natalie L Berntsen; Olav Klingenberg; Brian D Juran; Maria Benito de Valle; Björn Lindkvist; Konstantinos N Lazaridis; Kirsten Muri Boberg; Tom H Karlsen; Johannes Roksund Hov Journal: Gastroenterology Date: 2015-02-02 Impact factor: 22.682
Authors: Keith D Lindor; Kris V Kowdley; Velimir A C Luketic; M Edwyn Harrison; Timothy McCashland; Alex S Befeler; Denise Harnois; Roberta Jorgensen; Jan Petz; Jill Keach; Jody Mooney; Carol Sargeant; Julie Braaten; Tamara Bernard; Debra King; Ellen Miceli; Jeff Schmoll; Tanya Hoskin; Prabin Thapa; Felicity Enders Journal: Hepatology Date: 2009-09 Impact factor: 17.425