IgG4 Related disease – a retrospective descriptive study highlighting Canadian experiences in diagnosis and management

BMC Gastroenterology

Research Article

Paul Kortan¹  Eric Lee¹  Gary May¹  Gideon M Hirschfield²  Kim Tae Kyoung³  Leyla Yazdi³  Korosh Khalili³  Catalina Coltescu⁴  Harshna Patel⁵ 

[1] Centre for Advanced Therapeutic Endoscopy and Endoscopic Oncology, St Michael’s Hospital, Toronto, ON, Canada;Centre for Liver Research and NIHR Biomedical Research Unit, Institute of Biomedical Research, The Medical School, University of Birmingham, 5th Floor, B15 2TT, Birmingham, UK;Department of Medical Imaging, University Health Network, Toronto, ON, Canada;Liver Centre, Toronto Western Hospital, University of Torontocpf, Toronto, ON, Canada;Liver Centre, Toronto Western Hospital, University of Torontocpf, Toronto, ON, Canada;The Scarborough Hospital, Scarborough, ON, Canada;

关键词: Autoimmune pancreatitis;    Sclerosing cholangitis;    IgG4;   

DOI  :  10.1186/1471-230X-13-168

 received in 2012-10-09, accepted in 2013-11-15,  发布年份 2013

来源: Springer

【 摘 要 】

BackgroundAppreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice.MethodsComparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review.Results66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n = 50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n = 21; b) Sclerosing cholangitis, n = 9; c) Sclerosing pancreatitis, n = 4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n = 7; e) Sclerosing cholangitis with hepatic pseudotumour, n = 3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n = 1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria.ConclusionOur descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.

【 授权许可】

Unknown   
© Patel et al.; licensee BioMed Central Ltd. 2013. This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.