title: Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Hallym University College of Medicine, Hallym University Sacred Heart Hospital, Anyang, South Korea.
authors.forename: Sung-Hoon
authors.initials: SH
authors.lastname: Moon
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea. mhkim@amc.seoul.kr.
authors.forename: Myung-Hwan
authors.initials: MH
authors.lastname: Kim
authors.affiliation: Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-Ro, Gangnam-Gu, Seoul, South Korea. jongk.lee@samsung.com.
authors.forename: Jong Kyun
authors.initials: JK
authors.lastname: Lee
authors.affiliation: Department of Clinical Epidemiology and Biostatistics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea.
authors.forename: Seunghee
authors.initials: S
authors.lastname: Baek
authors.affiliation: Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-Ro, Gangnam-Gu, Seoul, South Korea.
authors.forename: Young Sik
authors.initials: YS
authors.lastname: Woo
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Dong Hui
authors.initials: DH
authors.lastname: Cho
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Dongwook
authors.initials: D
authors.lastname: Oh
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Tae Jun
authors.initials: TJ
authors.lastname: Song
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Do Hyun
authors.initials: DH
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Sang Soo
authors.initials: SS
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Dong Wan
authors.initials: DW
authors.lastname: Seo
authors.affiliation: Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Sung Koo
authors.initials: SK
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D001650:Bile Duct Neoplasms / Q000175:diagnosis; D015415:Biomarkers / Q000097:blood; D018281:Cholangiocarcinoma / Q000175:diagnosis; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D015415:Biomarkers; D007074:Immunoglobulin G
keywords: Diagnosis; IgG4-related sclerosing cholangitis; Primary sclerosing cholangitis; Scoring system