Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis

• Published in: Journal of gastroenterology Vol. 52; no. 4; pp. 483 - 493
• Main Authors: Moon, Sung-Hoon, Kim, Myung-Hwan, Lee, Jong Kyun, Baek, Seunghee, Woo, Young Sik, Cho, Dong Hui, Oh, Dongwook, Song, Tae Jun, Park, Do Hyun, Lee, Sang Soo, Seo, Dong Wan, Lee, Sung Koo
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01.04.2017; Springer Nature B.V
• Subjects: Abdominal Surgery; Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Algorithms; Autoimmune Diseases - complications; Autoimmune Diseases - diagnosis; Autoimmune Diseases - pathology; Bile Duct Neoplasms - diagnosis; Biliary Tract; Biomarkers - blood; Cholangiocarcinoma - diagnosis; Cholangitis, Sclerosing - complications; Cholangitis, Sclerosing - diagnosis; Cholangitis, Sclerosing - pathology; Colorectal Surgery; Diagnosis, Differential; Female; Gastroenterology; Hepatology; Humans; Immunoglobulin G - blood; Male; Medicine; Medicine & Public Health; Middle Aged; Original Article—Liver; Pancreas; Surgical Oncology; Young Adult; IgG4-related sclerosing cholangitis; Primary sclerosing cholangitis; Diagnosis; Scoring system
• ISSN: 0944-1174; 1435-5922
• DOI: 10.1007/s00535-016-1246-5

Background Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. Methods Patients with IgG4-SC ( n  = 39) and PSC ( n  = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method. Results The scoring system in the final model included age (<30 years, 0 points; 30–39 years, 1 point; 40–49 years, 2 points; 50–59 years, 3 points; ≥60 years, 4 points), OOI (no, 0 points; yes, 3 points), and beaded appearance (yes, 0 points; no, 2 points). The patients were classified according to their total score into three categories: 0–4 points, probable PSC; 5–6 points, indicating diagnostic steroid trial; 7–9 points, probable IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986). Conclusions A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.