Abstract
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.
Highlights
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
In the absence of this, the diagnosis was made if patients had classical radiological features of Autoimmune pancreatitis (AIP) and an elevated serum IgG4 > 2 × upper limit of normal (ULN) (>1.72 g/L)
Given our selection criteria we report on type 1 autoimmune pancreatitis; the relative importance of type 2 autoimmune pancreatitis cannot be adequately addressed by our study; in our chart survey only 4 patients were provisionally identified that were potentially type 2 AIP and only limited clinical information on 2 was available, our focus being classic AIP
Summary
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. Autoimmune pancreatitis (AIP) is the umbrella term for a group of multi-system infiltrative and inflammatory relapsing and remitting conditions, not the least limited to pancreatic involvement, and for which clinical presentation is protean, and in which no single test is diagnostic [1,2,3]. Presenting as a multisystem fibro-inflammatory condition there are various distinctive clinical, radiological, serological and pathological features, which point towards the diagnosis, since no single uniform presentation predominates [6,7]. The diagnosis is reached in patients with painless obstructive jaundice secondary to an inflammatory pancreatic mass with biliary involvement. The repeated demonstration of elevated IgG4 levels in patients with PSC has raised the spectre of a possible sub-group of patients with PSC who might have a forme-fruste or missed diagnosis of AIP, and steroids in this setting has been discussed widely [12,13]
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