P260 Autoimmune pancreatitis in patients with Inflammatory Bowel Disease: results from a multicentre ECCO CONFER cases series

Abstract

Background Autoimmune pancreatitis (AIP) is an uncommon inflammatory disorder that may accompany inflammatory bowel disease (IBD). The clinical relevance of AIP-IBD coexistence, therapeutic strategies, and long-term outcomes are scarcely known. Methods In this ECCO COllaborative Network For Exceptionally Rare case reports project (ECCO-CONFER) we included cases of AIP diagnosed in patients with IBD. Data on the diagnostic criteria, clinical characteristics, and patient outcomes were retrospectively collected and analysed. Results The cohort included 82 patients [54% males, 77% with ulcerative colitis (UC), age at IBD and AIP diagnosis 32±16 and 35±16 years, respectively]. Of all AIP patients, 71% were classified as type 2, 21% as type 1, and 8% as undefined type. Detailed characteristics of the study group are shown in Table 1. In 57% of the individuals, IBD was diagnosed before AIP. In the remaining cases, IBD was diagnosed simultaneously (20%) or afterwards (23%). Half of the patients required immunomodulators and 43% biological therapy to control their IBD. Twenty-two percent of UC and 6% of Crohn’s disease (CD) required surgical intervention during a mean follow-up of 3.6±3.4 years. Histological confirmation was used in 38% of cases, while the remaining were diagnosed as probable AIP based on clinical, biochemical, and radiologic criteria. Magnetic resonance was the most frequently used imaging modality (61%). Eighty percent of patients received steroids for AIP. Thiopurines were used for AIP in 16 (19%) patients, 81% of them responded to treatment. One patient was successfully treated for AIP with infliximab. AIP complications occurred in 27%, including pancreatic exocrine insufficiency (19%) and diabetes (13%). Five patients underwent surgery due to suspected pancreatic cancer. Patients with complicated AIP were significantly older at AIP and IBD diagnosis, were more frequently males and presented with weight loss at AIP diagnosis, and had less frequently a family history of IBD (Table 1). Colonic IBD (i.e. UC, isolated colonic IBD-unclassified or colonic CD) was seen in a higher proportion of individuals with complicated vs benign AIP course (91% vs 33%, p<0.0001). At the end of follow-up, 75 (91%) and 73 (89%) patients were in remission of AIP and IBD, respectively. No IBD or AIP-related deaths were reported. Conclusion In this large international cohort of patients with concomitant AIP-IBD, most patients have type 2 AIP and colonic IBD. AIP course is relatively benign, and long-term outcomes are favourable. Older age, male gender, isolated colonic IBD location, and weight loss at AIP presentation were seen in higher proportions of patients with a complicated course of AIP.

Clinical features and long‐term outcomes of patients with type 2 autoimmune pancreatitis

“…The risk of endocrine and exocrine insufficiency was extremely low in non‐operated patients, suggesting that the pancreatic function is preserved over time in type 2 AIP patients. Similar data have been published by Eder and colleagues, reporting 20% exocrine insufficiency and 11% endocrine insufficiency in 96 patients with concomitant IBD and AIP (including type 1, 2 and NOS) 18 …”

Autoimmune pancreatitis—New evidence for clinical management strategies

“…This is particularly important as most patients responded well to steroids and the relapse rate was low. Endocrine and exocrine insufficiency (around 10%) was lower as compared to other recently published studies, 9 but increased in surgically resected patients. Notably, none of the patients developed PDAC during the observation period which is in line with existing literature, 9 providing evidence that a life‐long pancreas specific follow‐up may not be necessary in all AIP type‐2 patients.…”

Autoimmune pancreatitis associated with inflammatory bowel diseases: A retrospectively bidirectional case–control study in China

“…34 Additionally, older age at AIP diagnosis was associated with a complicated AIP course (OR 1.05, P = 0.008), while a family history of IBD (OR 0.1, P = 0.03) and a diagnosis of CD (OR = 0.2, P = 0.04) were associated with an uncomplicated course of AIP. 34 Seven patients with different infections, which might be related to the high prevalence of HBV, hepatitis C virus, and TB infections in China. [35][36][37] Moreover, we observed a significantly more frequent hospitalization in AIP-IBD patients compared to AIP controls, while no significant difference was observed in comparison to IBD controls.…”

“…The advanced age at the time of AIP diagnosis and the prolonged duration of AIP in our patient population may provide a plausible explanation for these differences. Meanwhile, a recent cohort study involving 96 AIP‐IBD patients demonstrated that during a mean follow‐up of 7 years, approximately one‐fourth of the patients experienced complications 34 . Additionally, older age at AIP diagnosis was associated with a complicated AIP course (OR 1.05, P = 0.008), while a family history of IBD (OR 0.1, P = 0.03) and a diagnosis of CD (OR = 0.2, P = 0.04) were associated with an uncomplicated course of AIP 34 .…”

“…Meanwhile, a recent cohort study involving 96 AIP‐IBD patients demonstrated that during a mean follow‐up of 7 years, approximately one‐fourth of the patients experienced complications 34 . Additionally, older age at AIP diagnosis was associated with a complicated AIP course (OR 1.05, P = 0.008), while a family history of IBD (OR 0.1, P = 0.03) and a diagnosis of CD (OR = 0.2, P = 0.04) were associated with an uncomplicated course of AIP 34 . Seven patients with different infections, which might be related to the high prevalence of HBV, hepatitis C virus, and TB infections in China 35–37 .…”