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Background Autoimmune pancreatitis (AIP) is rarely associated with inflammatory bowel disease (IBD). Long-term outcomes of AIP and IBD in patients with AIP-IBD coexistence and predictors of complicated AIP course are scarcely known. Methods An ECCO COllaborative Network For Exceptionally Rare case reports project (ECCO-CONFER) collected cases of AIP diagnosed in patients with IBD. Complicated AIP was defined as a composite of endocrine and/or exocrine pancreatic insufficiency, and/or pancreatic cancer. We explored factors associated with complicated AIP in IBD. Results We included 96 patients (53% males, 79% ulcerative colitis, 72% type 2 AIP, age at AIP diagnosis 35±16 years). The majority of Crohn’s disease (CD) cases (78%) had colonic/ileocolonic involvement. In 59%, IBD preceded AIP diagnosis, whereas 18% were diagnosed simultaneously. Advanced therapy to control IBD was used in 61% and 17% underwent IBD-related surgery. 82% of patients were treated with steroids for AIP, the majority of which (91%) responded to a single course of treatment. During a mean follow-up of 7 years, AIP complications occurred in 25/96 (26%) individuals. In a multivariate model, younger age at AIP diagnosis (OR=1.05, P=0.008), family history of IBD (OR=0.1, P=0.03) and CD diagnosis (OR=0.2, P=0.04) were associated with uncomplicated AIP course. No IBD or AIP-related deaths occurred. Conclusions 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, however, one-quarter develop pancreatic complications. Age, familial history of IBD and CD may predict uncomplicated AIP course.

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Objectives Gut inflammation commonly occurs in axial spondyloarthritis (axSpA), and is linked to disease activity and outcome. Given the role of IgA in mucosal immunity, we explored the association between anti-CD74 IgA antibodies, gut inflammation and axSpA. Methods Anti-CD74 IgA was measured by ELISA in serum samples of axSpA patients (fulfilling the 2009 ASAS classification criteria). A group of fibromyalgia (FM) and rheumatoid arthritis (RA) patients served as non-inflammatory and inflammatory controls. Newly diagnosed axSpA patients underwent ileocolonoscopy; mucosal biopsies were histopathologically assessed as normal, acute or chronically inflamed. Optimal anti-CD74 IgA cut-off values were determined with a receiver operating characteristics (ROC) curve. Results AxSpA patients (n = 281) showed higher anti-CD74 IgA levels (mean±SD 18.8 ± 12.4 U/ml) compared with 100 FM patients (10.9 ± 5.0 U/ml, p< 0.001) and 34 RA patients (13.7 ± 9.6 U/ml, p= 0.02). The area under the ROC curve (AUC) for diagnosis (axSpA vs FM) was 0.70, providing a sensitivity of 60% and specificity of 87% (cut-off 15 U/ml). Antibody concentrations were not significantly different between axSpA patients with (n = 40) and without (n = 69) gut inflammation (p= 0.83), yielding an AUC of 0.51. Anti-CD74 IgA levels were not associated with degree of bone marrow oedema on MRI of the sacroiliac joints, CRP, or any other disease-specific feature such as the use of NSAIDs or biological treatment. Conclusion Serum anti-CD74 IgA is a potentially useful diagnostic biomarker for axSpA. However, antibody levels do not correlate with any phenotypical feature, including microscopic gut inflammation, suggesting this to be a disease-specific rather than an inflammatory marker.

Background Ustekinumab (UST) is increasingly used in Belgium for moderate to severe Crohn’s disease (CD). The aim of the current study was to describe the real-life experience with UST in our tertiary centre. Methods A retrospective study was performed in patients with CD who were started on UST between December 2017 and August 2019. All patients received an initial intravenous (IV) dose of 6 mg/kg body-weight, followed by 8-weekly 90 mg subcutaneous UST. The clinical and endoscopic response was assessed by the physician after induction and during the maintenance phase. Results In total, 67 patients were included, of which the majority was refractory to anti-TNF and/or vedolizumab (Table 1). The median duration of treatment was 15 months (IQR 7–25). The clinical and endoscopic response is shown in Table 2. UST was discontinued in 16 patients (23.9%) after a median of 27.5 weeks (IQR 12.3–52.8). Reasons for discontinuation were a loss of response (LOR), including 5 patients who were in need of surgery (n = 10), primary non response (n = 3), malignancy (n = 1), adverse event (n = 1) and patients’ wish (n = 1). In 29 patients (43.3%) optimisation of UST was necessary due to partial response (n = 13) or due to LOR (n = 16). The optimisation consisted of an IV re-induction in 2 patients, shortening of the dosage interval in 16 patients and a combination of both in 11 patients. The effect of optimisation could be assessed in 22 patients: 10 patients regained a good clinical response and 5 patients attained clinical remission after optimisation. In 7 patients UST was stopped despite optimisation, most often due to persistent LOR. Conclusion In this tertiary population of refractory CD patients, treatment with UST resulted in a clinical response in more than 70% of patients. Of note, 43.3% needed optimisation, with a good clinical response in almost half of the cases. The endoscopic response in this preliminary analysis was modest.

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