Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment.

BACKGROUND & AIMS: Autoimmune pancreatitis (AIP) is an immune-mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large pan-European cohort and study the effectiveness of current treatment regimens. METHODS: We retrospectively analyzed adults diagnosed since 2005 with type 1 or not-otherwise-specified AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using specific diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary end point was complete remission, defined as the absence of clinical symptoms and resolution of the index radiologic pancreatic abnormalities attributed to AIP. RESULTS: We included 735 individuals with AIP (69% male; median age, 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, whereas 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher (≥0.4 mg/kg/day) corticosteroid doses were no more effective than lower (<0.4 mg/kg/day) doses (odds ratio, 0.428; 95% confidence interval, 0.054-3.387) and neither was a starting dose duration >2 weeks (odds ratio, 0.908; 95% confidence interval, 0.818-1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (odds ratio, 0.639; 95% confidence interval, 0.427-0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid-tapering duration, induction treatment duration, and total cumulative dose. CONCLUSIONS: Patients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens.

A case report of pancreatic exocrine insufficiency in a patient with Parkinson's disease: A coincidence or is there more to it than meets the eye?

Pancreatic exocrine insufficiency (PEI) is an under-diagnosed condition. Untreated PEI can result in developing gastrointestinal symptoms and long-term complications including weight loss, nutrient deficiencies, sarcopenia and osteoporosis. Current best practice recommends testing for PEI in certain disorders including chronic pancreatitis, cystic fibrosis, pancreatic cancer and post-pancreatic surgery. However, there is increasing evidence that PEI is associated with a number of conditions in addition to the aforementioned diseases. These 'at-risk' conditions are a heterogeneous group of diseases, for example, diabetes mellitus, people living with human immunodeficiency virus, high alcohol intake, and coeliac disease. The pathophysiology of some of 'at-risk' conditions is becoming increasingly recognised; therefore, the list of associated conditions are in evolving process. We present a case of a 60-year-old male with Parkinson's disease and persistent abdominal pain who was found to have low faecal elastase levels indicative of severe PEI. His past medical history included none of the known risk factors for PEI. After examining the literature, we report a similar pathophysiological process underlying the development of pancreatitis and Parkinson's disease which is dysfunction of the Unfolded Protein Response. We suggest further research to assess the prevalence of PEI in the population of patients with Parkinson's disease.

What is the clinical significance of low serum amylase? Systematic review of the conditions associated with low serum amylase.

Objective: Most studies have assessed the impact of elevated serum amylase levels in clinical practice, but only a few have investigated the significance of low serum amylase. We therefore, aimed to review the literature to understand the conditions associated with low serum amylase and its clinical relevance. Method: This systematic review was performed in accordance with the criteria established in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The search was conducted on Medline and Embase databases until November 2022. After identifying relevant titles, abstracts were read and data of eligible articles retrieved. The conditions associated with low serum amylase were evaluated. The quality of the studies was assessed using the Newcastle-Ottawa Score. Results: Our search strategy identified 19 studies including a total of 15 097 patients for systematic review. All the studies were observational including two studies which used secretin-induced test. The main conditions associated with low serum amylase were diabetes mellitus (n=9), metabolic syndrome (n=3), chronic pancreatitis (CP) (n=3), non-alcoholic fatty liver disease (n=2) and obesity (n=1). Low serum amylase showed a high specificity (94%) with low sensitivity (38.7%-59%) in diagnosing chronic pancreatitis. Conclusion: This systematic review revealed a unique insight into the relevance of low serum amylase in clinical practice. Low serum amylase can be a useful adjunct test in the assessment of patients with CP, pancreatic exocrine insufficiency, diabetes mellitus and metabolic syndrome.