Sung-Hoon Moon1, Myung-Hwan Kim2, Jong Kyun Lee3, Seunghee Baek4, Young Sik Woo5, Dong Hui Cho6, Dongwook Oh6, Tae Jun Song6, Do Hyun Park6, Sang Soo Lee6, Dong Wan Seo6, Sung Koo Lee6. 1. Department of Internal Medicine, Hallym University College of Medicine, Hallym University Sacred Heart Hospital, Anyang, South Korea. 2. 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. 3. Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-Ro, Gangnam-Gu, Seoul, South Korea. jongk.lee@samsung.com. 4. Department of Clinical Epidemiology and Biostatistics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea. 5. Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-Ro, Gangnam-Gu, Seoul, South Korea. 6. Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea.
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.
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.
Entities:
Keywords:
Diagnosis; IgG4-related sclerosing cholangitis; Primary sclerosing cholangitis; Scoring system
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