Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan

Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4‐related sclerosing cholangitis (IgG4‐SC) might be misdiagnosed as PSC. Since the clinical diagnos...

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Published inJournal of hepato-biliary-pancreatic sciences Vol. 21; no. 1; pp. 43 - 50
Main Authors Tanaka, Atsushi, Tazuma, Susumu, Okazaki, Kazuichi, Tsubouchi, Hirohito, Inui, Kazuo, Takikawa, Hajime
Format Journal Article
LanguageEnglish
Published Japan Blackwell Publishing Ltd 01.01.2014
Wiley Subscription Services, Inc
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Online AccessGet full text
ISSN1868-6974
1868-6982
1868-6982
DOI10.1002/jhbp.50

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Abstract Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4‐related sclerosing cholangitis (IgG4‐SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4‐SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4‐SC lacking pancreatic involvement. Methods The design was a questionnaire‐based, multi‐center retrospective study. The enrolled subjects were patients with PSC and IgG4‐SC without pancreatic involvement diagnosed after 2005. Results We enrolled 197 PSC and 43 IgG4‐SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4‐SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4‐SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4‐SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4‐SC was considerably better than that of PSC. Conclusion We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
AbstractList Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4‐related sclerosing cholangitis (IgG4‐SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4‐SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4‐SC lacking pancreatic involvement. Methods The design was a questionnaire‐based, multi‐center retrospective study. The enrolled subjects were patients with PSC and IgG4‐SC without pancreatic involvement diagnosed after 2005. Results We enrolled 197 PSC and 43 IgG4‐SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4‐SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4‐SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4‐SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4‐SC was considerably better than that of PSC. Conclusion We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement. The design was a questionnaire-based, multi-center retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005. We enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4-SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC. We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement.BACKGROUNDWe previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement.The design was a questionnaire-based, multi-center retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005.METHODSThe design was a questionnaire-based, multi-center retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005.We enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4-SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC.RESULTSWe enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4-SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC.We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.CONCLUSIONWe confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC patients, yet patients with IgG4-related sclerosing cholangitis (IgG4-SC) might be misdiagnosed as PSC. Since the clinical diagnostic criteria of IgG4-SC were established in 2012, we again conducted a nationwide survey to investigate the characteristics of PSC and IgG4-SC lacking pancreatic involvement. Methods The design was a questionnaire-based, multi-center retrospective study. The enrolled subjects were patients with PSC and IgG4-SC without pancreatic involvement diagnosed after 2005. Results We enrolled 197 PSC and 43 IgG4-SC patients without pancreatic lesions. The male dominance was significantly evident in IgG4-SC (P = 0.006). In patients with PSC, two peaks in age distribution were clearly observed. IgG4-SC was not detected in any patient younger than 45 years of age. At presentation, serum albumin and IgM were significantly higher in PSC, while serum IgG and IgG4 were significantly elevated in IgG4-SC. Inflammatory bowel disease (IBD) was detected in only 68/197 PSC patients (34%). The prognosis of IgG4-SC was considerably better than that of PSC. Conclusion We confirmed several interesting clinical details of PSC in Japanese patients: two peaks in the age distribution and lower prevalence of IBD.
Author Inui, Kazuo
Okazaki, Kazuichi
Tsubouchi, Hirohito
Takikawa, Hajime
Tazuma, Susumu
Tanaka, Atsushi
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  organization: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Tokyo, 173-8605, Itabashi-ku, Japan
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Keywords Inflammatory bowel diseases
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Autoimmune pancreatitis
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– reference: Molodecky NA, Kareemi H, Parab R, Barkema HW, Quan H, Myers RP, et al. Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis. Hepatology. 2011;53:1590-1599.
– reference: Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706-715.
– reference: Takikawa H, Takamori Y, Tanaka A, Kurihara H, Nakanuma Y. Analysis of 388 cases of primary sclerosing cholangitis in Japan; Presence of a subgroup without pancreatic involvement in older patients. Hepatol Res. 2004;29:153-159.
– reference: Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, et al. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol. 2004;28:1193-1203.
– reference: Karlsen TH, Boberg KM. Update on primary sclerosing cholangitis. J Hepatol. 2013;59:571-582.
– reference: Takikawa H, Manabe T. Primary sclerosing cholangitis in Japan-analysis of 192 cases. J Gastroenterol. 1997;32:134-137.
– reference: Broome U, Olsson R, Loof L, Bodemar G, Hultcrantz R, Danielsson A, et al. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut. 1996;38:610-615.
– reference: Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas. 2006;32:229.
– reference: Lindkvist B, Benito de Valle M, Gullberg B, Bjornsson E. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology. 2010;52:571-577.
– reference: Farrant J, Hayllar K, Wilkinson M, Karani J, Portmann B, Westaby D, et al. Natural history and prognostic variables in primary sclerosing cholangitis. Ganstroenterology. 1991;100:1710-1717.
– reference: Card TR, Solaymani-Dodaran M, West J. Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol. 2008;48:939-944.
– reference: Tanaka A, Tazuma S, Okazaki K, Tsubouchi H, Inui K, Takikawa H. A nation-wide survey of sclerosing cholangitis. Tando. 2013;27:176-187 (in Japanese with English abstract).
– reference: Hamano H, Umemura T, Uehara T, Kawa S, Kiyosawa K. IgG4-related sclerosing cholangitis should be included as an exclusion criterion for the diagnosis of primary sclerosing cholangitis. Am J Gastroenterol. 2007;102:691-692.
– reference: Bambha K, Kim WR, Talwalkar J, Torgerson H, Benson JT, Therneau TM, et al. Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community. Gastroenterology. 2003;125:1364-1369.
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Snippet Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of...
We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of Japanese PSC...
Background We previously conducted nationwide surveys for primary sclerosing cholangitis (PSC) in Japan, and demonstrated several characteristic features of...
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SubjectTerms Adult
Aged
Autoimmune Diseases - complications
Autoimmune Diseases - epidemiology
Autoimmune Diseases - therapy
Autoimmune pancreatitis
Cholangitis, Sclerosing - complications
Cholangitis, Sclerosing - epidemiology
Cholangitis, Sclerosing - therapy
Female
Humans
Immunoglobulin G - immunology
Inflammatory bowel disease
Inflammatory bowel diseases
Japan
Male
Middle Aged
Nationwide survey
Prednisolone
Prognosis
Retrospective Studies
Surveys and Questionnaires
Ursodeoxycholic acid
Title Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan
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https://onlinelibrary.wiley.com/doi/abs/10.1002%2Fjhbp.50
https://www.ncbi.nlm.nih.gov/pubmed/24353071
https://www.proquest.com/docview/1706900464
https://www.proquest.com/docview/1490745126
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