Introduction Improvement in jaundice and pancreatic enlargement with prednisolone is a reliable and characteristic feature of autoimmune pancreatitis (AIP). The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as this has not been well defined.
Methods We report a single-centre cohort study of all patients diagnosed with AIP from 2004 to 2009. Diagnosis was based on the HISORt criteria, while assessment of pancreatic morphology was made pre and post-treatment, by CT and/or MRI, according to clinical need. Exocrine and endocrine function was measured by stool faecal elastase 1 (FE1) and plasma glucose/HbA1c.
Results Forty-one patients (33M/F8; median age 62 years, range 29–83) were included. Median follow-up to date is 35 months (range 1–71). At diagnosis, a pancreatic mass/diffuse swelling was noted in 36/41 (88%), serum IgG4 was elevated (>1.3 g/l) in 22/41 (54%), and 30/41 (73%) had an immunohistological diagnosis, with >10 IgG4-positive plasma cells/high power field (n=15 pancreas, n=8 liver, n=8 ampulla, n=2 salivary gland, n=2 renal). 30/41 (73%) had IgG4-associated cholangitis, 14/41 (34%) had other organ involvement (n=7 renal, n=3 salivary gland, n=2 retroperitoneal fibrosis, n=2 neurological). Seven (17%) of 41 patients had previously undergone pancreatobiliary surgery for presumed pancreatic cancer (n=3 Whipple resection, n=4 biliary bypass). 38/41 (93%) patients were treated with a tapering oral prednisolone regimen, which was ceased at a median of 6 months (range 2.5–70.9 months). An initial clinical, radiological, and biochemical response was seen in all patients. Relapse following disease remission or failure to wean steroids occurred in 19/41 (46%), and of these patients 6/19 were treated with steroids, and 13/19 were treated with prednisolone 30 mg and azathioprine 2 mg/kg daily. Post-steroid pancreatic atrophy was seen in 23/41 (56%). Low FE1 (<200 μg/g of stool) was noted in 23/41 (56%). 15/41 (37%) patients were diabetic, while 9/41 (22%) have reduced bone mineral density (n=5 osteopaenia, n=4 osteoporosis). Three patients have died during follow-up (n=1 liver failure, n=1 encephalitis, n=1 cholangiocarcinoma).
Conclusion While a favourable initial response to steroids is predictable in patients with a definitive diagnosis of AIP, loss of pancreatic structure and function is common, and the disease is associated with long-term morbidity and mortality. It will be difficult to clearly define the impact of treatment on the outcome of pancreatic and extra-pancreatic disease without a placebo controlled trial.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.