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

BMC Gastroenterology

Gideon M Hirschfield²  Catalina Coltescu¹  Paul Kortan⁵  Gary May⁵  Eric Lee⁵  Leyla Yazdi⁴  Kim Tae Kyoung⁴  Korosh Khalili⁴  Harshna Patel³ 

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

关键词: IgG4;    Sclerosing cholangitis;    Autoimmune pancreatitis;   

Others  :  856839
DOI  :  10.1186/1471-230X-13-168

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

【 摘 要 】

Background

Appreciating 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.

Methods

Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review.

Results

66 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.

Conclusion

Our 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】

   
2013 Patel et al.; licensee BioMed Central Ltd.

【 图 表 】

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