Autoimmune Pancreatitis in Patients with Inflammatory Bowel Disease: A Real-World Multicentre Collaborative ECCO CONFER Study.

BACKGROUND: Autoimmune pancreatitis [AIP] is rarely associated with inflammatory bowel disease [IBD]. The long-term outcomes of AIP and IBD in patients with coexisting AIP-IBD and predictors of complicated AIP course have rarely been reported. 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 ±â€…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. In total, 82% of patients were treated with steroids for AIP, the majority of whom [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, older age at AIP diagnosis was associated with a complicated AIP course (odds ratio [OR] = 1.05, p = 0.008), whereas family history of IBD [OR = 0.1, p = 0.03], and CD diagnosis [OR = 0.2, p = 0.04] decreased the risk of AIP complications. 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, but one-quarter develop pancreatic complications. Age, familial history of IBD, and CD may predict uncomplicated AIP course.

The usefulness of selected sequences of magnetic resonance imaging in a diagnostic model for Crohn's disease.

Introduction: We hypothesized that, based on magnetic resonance enterography (MRE) and the apparent diffusion coefficient (ADC), measured in the affected parts of the intestine, it is possible to effectively differentiate active and chronic phases of Crohn's disease. Aim: To create a multidimensional diagnostic model for differentiating between the phases of Crohn's disease. Material and methods: This study included 125 patients - 55 women (aged 19 to 66 years) and 70 men (aged 12 to 67 years) - who underwent MRE and ADC measurement for the first time. Results: The group of potential explanatory variables comprised 11 variables, including the thickness and length of the occupied section, number of lymph nodes, layered bowel wall enhancement, total transitions on fat tissue, and features of restricted diffusion. The final discrimination model was based on 2 variables: ADC (A) and layered bowel wall enhancement (W). Active Crohn's disease was defined as -6.339 + 4.747 × W + 0.008 × A, while chronic Crohn's disease was defined as -11.365 + 2.812 × W + 0.012 × A. Definitive diagnosis was based on histological examination of material collected during ileocolonoscopy in 96 patients, surgery with subsequent histopathological examination in 17 patients, and capsule endoscopy in 12 patients. Conclusions: The predictive model described here could identify the active form of Crohn's disease with a probability of 93.06% and the chronic form with a probability of 75.57%. The use of classic MRE layered bowel wall enhancement and a DWI-based ADC metric eliminates the main shortcomings of both approaches.

Is hepatotropic contrast enhanced MR a more effective method in differential diagnosis of hemangioma than multi-phase CT and unenhanced MR?

BACKGROUND: Cavernous hemangiomas are the most frequent neoplasms of the liver and in routine clinical practice they often need to be differentiated from malignant tumors and other benign focal lesions. The purpose of this study is to evaluate whether diagnostic accuracy of magnetic resonance imaging (MRI) of hepatic hemangiomas, showing atypical pattern on US, improves with the use of Gd-BOPTA in comparison with contrast-enhanced multi-phase computed tomography (CT). METHODS: 178 consecutive patients with ambiguous hepatic masses showing atypical hyperechoic pattern on grey-scale US, underwent unenhanced and contrast-enhanced multi-phase multi-detector CT and MR (1.5T) with the use of liver-specific contrast medium gadobenate dimeglumine (Gd-BOPTA). After intravenous contrast administration arterial (HAP), venous-portal (PVP), equilibrium phases (EP) both in CT and MR and additionally hepatobiliary phase (HBP) in MR were obtained. 398 lesions have been detected including 99 hemangiomas and 299 other lesions. RESULTS: In non-enhanced MDCT examination detection of hemangiomas was characterized by sensitivity of 76%, specificity of 90%, PPV of 71%, NPV of 92% and accuracy of 86%.Non-enhanced MR examination showed sensitivity of 98%, specificity of 99%, PPV of 99%, NPV of 99% and accuracy of 99%.After intravenous administration of contrast medium in MR the mentioned above parameters did not increase significantly. CONCLUSION: Gd-BOPTA-enhanced MR in comparison with unenhanced MRI does not improve diagnostic accuracy in discriminating hemangiomas that show non-specific appearance in ultrasound examination. Unenhanced MR as a method of choice should directly follow US in course of diagnostic algorithm in differentiation of hemangiomas from other liver tumors.

Characteristics of patients with moderate-to-severe ulcerative colitis treated with vedolizumab: results from a Polish multicenter, prospective, observational real-life study (the POLONEZ study).

BACKGROUND: Vedolizumab, a humanized antibody targeting the α4ß7 integrin, was proven to be effective in the treatment of moderate-to-severe ulcerative colitis (UC) in randomized clinical trials. The aim of the POLONEZ study is to determine the demographic and clinical characteristics of the patients with UC treated with vedolizumab within the scope of the National Drug Program in Poland and to assess the real-world effectiveness and safety of vedolizumab in the study population. Here we report the demographic and clinical characteristics of these patients. METHODS: This prospective study included adult patients eligible for UC treatment with vedolizumab who were recruited from 12 centers in Poland between February and November 2019. Collected data included sex, age, disease duration, presence of extraintestinal manifestations or comorbidities, status of previous biologic treatment, and current concomitant treatment. Disease extent was determined according to the Montreal classification, and disease activity was measured with the Mayo Score. RESULTS: A total of 100 (55 biologic-naïve and 45 biologic-exposed) patients were enrolled in the study (51% female, median age 35 years). Among biologic-exposed patients (mostly infliximab-treated), 57% had failed to respond to the therapy. The disease duration was significantly shorter in biologic-naïve (median 5 years) than in biologic-exposed (8 years, p = 0.004) or biofailure patients (7 years, p = 0.04). In the overall population the median Total Mayo Score was 10. Disease extent and activity were similar between the subgroups. CONCLUSIONS: Our study indicates that patients treated with vedolizumab in Poland receive the drug relatively early after UC diagnosis, but their disease is advanced. More than half of the patients had not been treated with biologic drugs before initiating vedolizumab. The study was registered in ENCePP database (EUPAS34119). LAY SUMMARY: Characteristics of patients treated for ulcerative colitis with vedolizumab in Poland Treatment of moderate-to-severe ulcerative colitis (UC) with the integrin antagonist vedolizumab became available within the Polish National Drug Program (NDP) in 2018. In this study, for the first time, we provide detailed demographic and clinical characteristics of 100 patients (median age 35 years, 51% female) treated with vedolizumab in Poland, of whom 55 were biologic-naïve and 45 biologic-exposed. The median duration of disease was 6 years. The disease duration was shorter in biologic-naïve than in biologic-exposed patients. Most patients were affected by extensive colitis (52%) or left-sided colitis (42%). Median disease activity was 10 according to the Total Mayo Score. Sixty-eight patients received concomitant systemic corticosteroids and 45 patients received immunomodulators. Our findings indicate that Polish patients receiving vedolizumab have a high disease activity and are treated relatively early after UC diagnosis. This might be due to the criteria for inclusion of a patient in the NDP.

Interstitial and Granulomatous Lung Disease in Inflammatory Bowel Disease Patients.

BACKGROUND: Interstitial lung [ILD] disease and granulomatous lung disease [GLD] are rare respiratory disorders that have been associated with inflammatory bowel disease [IBD]. Clinical presentation is polymorphic and aetiology is unclear. METHODS: This was an ECCO-CONFER project. Cases of concomitant ILD or GLD and IBD, or drug-induced ILD/GLD, were collected. The criteria for diagnosing ILD and GLD were based on definitions from the American Thoracic Society and the European Respiratory Society and on the discretion of reporting clinician. RESULTS: We identified 31 patients with ILD. The majority had ulcerative colitis [UC] [n = 22]. Drug-related ILD was found in 64% of these patients, 25 patients [80.6%] required hospitalisation, and one required non-invasive ventilation. The causative drug was stopped in all drug-related ILD, and 87% of patients received systemic steroids. At follow-up, 16% of patients had no respiratory symptoms, 16% had partial improvement, 55% had ongoing symptoms, and there were no data in 13%. One patient was referred for lung transplantation, and one death from lung fibrosis was reported. We also identified 22 GLD patients: most had Crohn's disease [CD] [n = 17]. Drug-related GLD was found in 36% of patients and 10 patients [45.4%] required hospitalisation. The causative drug was stopped in all drug-related GLD, and 81% of patients received systemic steroids. Remission of both conditions was achieved in almost all patients. CONCLUSIONS: ILD and GLD, although rare, can cause significant morbidity. In our series, over half of cases were drug-related and therefore focused pharmacovigilance is needed to identify and manage these cases.

Perianal fistulas in Crohn's disease: MRI diagnosis and surgical planning: MRI in fistulazing perianal Crohn's disease.

Crohn's disease is a chronic, transmural inflammatory process of the gastrointestinal tract. It often affects the colon with the perianal area. The most common intestinal manifestations include external and/or internal fistulas and abscesses. Assessment of the activity of perianal fistulas in the course of Crohn's disease seems to be an important factor influencing therapeutic approach. Fistula's activity is evaluated by such methods as magnetic resonance imaging, anal ultrasound and examination under anaesthesia. Usefulness of imaging methods in the diagnosis of fistulas still remains to be defined.MRI is used to present a wide spectrum of perianal fistulazing Crohn's disease. Additionally, it is an important instrument revealing location, extent and severity of inflammation. It is also very helpful to detect clinically silent sepsis related to small, local inflammation. The most common method used in MR imaging to assess topography of a fistula's track, is Parks' classification.Clinical indications to MRI may include follow-up studies of a diagnosed disease, classification of fistulas' subtypes in the course of Crohn's disease, determination of the extent of fistulas' tracts and spread of an inflammatory process what can guide surgical procedures.