Dataset Information
IgG4 related disease - a retrospective descriptive study highlighting Canadian experiences in diagnosis and management.
Ontology highlight
ABSTRACT: 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.
SUBMITTER: Patel H
PROVIDER: S-EPMC3878912 | biostudies-literature | 2013 Dec
REPOSITORIES: biostudies-literature
Publications
IgG4 related disease - a retrospective descriptive study highlighting Canadian experiences in diagnosis and management.
BMC gastroenterology 20131209
<h4>Background</h4>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.<h4>Methods</h4>Comparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review.<h4>Results</h4>66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifia ...[more]