OBJECTIVE: The aim of this study is to evaluate the ability of magnetic resonance enterography global score (MEGS) to diagnose the activity of pediatric Crohn's disease (CD) and its correlation with endoscopic activity score.
MATERIALS AND METHODS: 70 pediatric CD patients (between the ages of 6 and 17) were enrolled who underwent ileocolonoscopy and magnetic resonance enterography (MRE) within 7 days. The simplified endoscopic activity score for Crohn's disease (SES-CD) and MEGS were acquired in the terminal ileum. Sensitivity and specificity of MEGS for detection disease activity against SES-CD was compared using the McNemar test. The correlation between MEGS and SES-CD was assessed by Spearman's rank estimation. The diagnostic accuracy of MEGS for active disease defined by SES-CD was calculated. Receiver operating characteristic curves (ROC) were constructed.
RESULTS: Fifty-two pediatric CD patients (median age, 12 years old; 28 girls, 24 boys) were included. The incidence of upper gastrointestinal (GI) tract (23%) involvement and perianal lesions (42%) is high in pediatric Crohn's patients, and most of them suffer from internal hemorrhoids (86.5%). MEGS showed strong correlation to SES-CD (r = 0.70, P < 0.001). With endoscopic as the standard of reference, the MEGS had a high accuracy for the detection of inflammation (area under the ROC curve (AUC) of 0.89, sensitivity 0.95 and specificity 0.82) and for disease activity (AUC of 0.81, sensitivity 0.88 and specificity 0.75) in the terminal ileum.
CONCLUSION: Pediatric Crohn's disease is unique. Our study has shown a good correlation between MEGS and endoscopy activity score with equal diagnostic efficacy. MEGS is a promising method to assess disease activity and perhaps be a valuable tool in following therapeutic changes.
BACKGROUND & AIMS: Mucosal healing (MH) has become a goal of therapy for Crohn's disease (CD), but frequent endoscopies are not feasible. We aimed to develop and validate a non-invasive index to assess mucosal inflammation in children with CD.
METHODS: We collected data from the multi-center prospective ImageKids study, in which children with CD underwent ileocolonoscopy with magnetic resonance enterography. We investigated the association of pediatric CD activity index (PCDAI) items and laboratory test results with the simple endoscopic score for CD (SESCD). We used these data in a blended mathematical judgmental clinimetric approach to develop a weighted categorized index to identify children with CD who have MH, which we called the MINI index. We validated the index using data from 3 independent patient cohorts. The derivation and validation cohorts included 154 and 168 children, respectively (age 14.1 ± 2.5 years and 14.2 ± 3.9 years), of whom 16% and 36% had MH (defined as SESCD<3).
RESULTS: In multivariable models, the stooling item of the PCDAI, erythrocyte sedimentation rate, and level of fecal calprotectin were associated with SESCD (all P < .05). We added data on level of C-reactive protein to develop the MINI index. MINI scores below 8 identified children with MH with 88% sensitivity and 85% specificity in the derivation cohort and with 84% sensitivity and 87% specificity in the validation cohorts. Ninety percent of the patients in the validation cohort with scores of 8 or more had active mucosal inflammation, yet 78% of patients with scores below 8 had MH. Scores below 6 increase the positive predictive value to 86%.
CONCLUSIONS: We developed an index to non-invasively assess mucosal inflammation in children with CD. This index, identifies children with MH with high sensitivity and specificity. The added benefit of MINI over measurement of fecal calprotectin was small but significant, especially for patients with concentrations of fecal calprotectin from 100 to 599 μg/g. ClinicalTrials.gov no: NCT01881490.
AIM: Aim of this retrospective study is to evaluate the response to therapy in Crohn's disease (CD) patients studied by MR Enterography (MRE) in comparison with Harvey Bradshaw Index (HBI).
METHODS: One hundred and sixty patients with histological proved CD have undergone MRE in the last years. Forty-six patients who repeated MRE after medical therapy within six months were selected for the study. Magnetic Resonance Index of Activity (MaRIA) was evaluated for each patient and used to define the MR judgment. In MRE we also evaluated wall thickening, longitudinal extension of wall thickening, presence of stratified mural hyperenhancement and extraintestinal signs. The clinical response to therapy was judged based on HBI and classified as improved, worsened or stable disease. Clinical judgment was correlated with MRE findings and the agreement was analysed using the Cohen Kappa test.
RESULTS: Among 46 enrolled patients, 18 (39%) improved clinically, 4 (10%) worsened, 24 (51%) remained stable. MR judgment was in agreement with clinical assessment in 33 patients (72%), showing moderate significant concordance (Kappa = 0.49; p < 0.01). No agreement was observed in 13 (28%) patients. Moreover, clinical improvement was significantly correlated to reduction of wall thickening, reduction of longitudinal extension of the disease and reduction of engorged vasa recta (p < 0.05). Worsening conditions were significantly correlated to increased wall thickening (p = 0.05).
CONCLUSIONS: MRE is useful in evaluating the response to therapy in CD patients.
Endoscopic assessment is currently the criterion standard for the diagnosis and assessment of mucosal disease activity, prognosis and monitoring for dysplasia, and assessment of response to therapy. Wider appreciation of the potential disconnect between symptoms and objective measures of disease activity and evidence that uncontrolled inflammation may lead to progressive intestinal injury and irreversible bowel damage with adverse events has led to the concept of treating to target. Treating to target is defined as treating patients with high risk for disease progression early to prevent or limit intestinal injury or disability. Endoscopic remission (mucosal healing) has emerged as a key goal of therapy. Although there are no currently validated definitions of endoscopic mucosal remission, the use of endoscopic scoring systems add uniformity and objectivity and aid standardization with reporting of mucosal appearance, augmenting clinical decision making. A plethora of scoring systems exist to define activity, response, and remission in both Crohn's disease and ulcerative colitis. In this review, we discuss the most commonly used endoscopic scoring systems and proposed definitions of response and remission, and how they can be integrated into a treat-to-target approach to optimize patient outcomes.
BACKGROUND AND AIMS: Inflammatory bowel diseases [IBD] are disabling disorders. The IBD-Disability Index [IBD-DI] was developed for quantifying disability in IBD patients but is difficult to use. The IBD-Disk is a visual adaptation of the IBD-DI. It has not been validated yet. The main objectives were to validate the IBD-Disk and to assess the clinical factors associated with a change in the score and its variability over time.
METHODS: From May 2018 to July 2019, IBD patients from three university-affiliated hospitals responded twice to both IBD-Disk and IBD-DI at 3-12 month intervals. Validation included concurrent validity, reproducibility, and internal consistency. Mean IBD-Disk scores were compared according to clinical factors. Variability was assessed by comparing scores between baseline and follow-up visits.
RESULTS: A total of 447 patients [71% Crohn's disease, 28% ulcerative colitis] were included in the analysis at baseline and 265 at follow-up. There was a good correlation between IBD-Disk and IBD-DI [r = 0.75, p <0.001]. Reproducibility was excellent [intra-class correlation coefficient = 0.90], as well as internal consistency [Cronbach's α = 0.89]. The IBD-Disk was not influenced by IBD type but was associated with female gender and physician global assessment. Extra-intestinal manifestations, history of resection, elevated C-reactive protein and faecal calprotectin also tended to be associated with higher disability. The IBD-Disk score decreased in patients becoming inactive over time.
CONCLUSIONS: This study validated the IBD-Disk in a large cohort of IBD patients, demonstrating that it is a valid and reliable tool for quantifying disability for both CD and UC.
Crohn disease and ulcerative colitis are the two main types of inflammatory bowel disease. High rates of these conditions are seen in Australasian children - furthermore, increasing rates have been evident in recent years. Children can present with typical symptoms of abdominal pain, diarrhoea, haematochezia and/or weight loss. Atypical presentations (such as skin lesions or isolated short stature) can also occur: these may be associated with delays in the consideration and diagnosis of IBD. Initial steps in establishing a diagnosis of IBD include delineation of inflammatory markers exclusion of any other likely aetiology. Definitive diagnosis relies upon key endoscopic, histologic and radiological findings. Overall management of IBD encompasses care within a team-based, child and family-focused, multi-disciplinary setting.
BACKGROUND: A simplified magnetic resonance enterography (MRe) index (sMARIA) for Crohn's disease (CD) was recently developed and validated.
OBJECTIVE: Our aims were (a) to assess sMARIA's accuracy in a sample other than the validation cohort; (b) to evaluate its correlation with a simpler endoscopy index (SES-CD) and fecal calprotectin (FC); and (c) to assess the need of an expert radiologist to reliably use sMARIA.
METHODS: Patients with CD who underwent MRe, ileocolonoscopy and FC within 2-4 weeks had their MRe retrospectively reviewed by two blinded raters. Disease activity was evaluated through sMARIA, SES-CD and FC. sMARIA's accuracy, indices correlation, and interrater reliability were assessed.
RESULTS: In total, 84 patients were included, comprising 420 intestinal segments evaluations. sMARIA ≥1 accurately identified segments with active disease (90% sensitivity, 98% specificity; area under the curve 0.94, 95% confidence interval (CI) 0.91-0.97; p < 0.01). sMARIA correlated with endoscopy, both for ileal and colonic segments (R = 0.94 and R = 0.82; p < 0.01). Per patient, there was a strong correlation between sMARIA, endoscopy (R = 0.95; p < 0.01) and FC (R = 0.91; p < 0.01). Interrater agreement was excellent (intraclass correlation coefficient 0.95; 95% CI 0.94-0.96; p < 0.01).
CONCLUSION: sMARIA accurately measured CD activity using SES-CD as standard of reference, and exhibited high correlation with a simple endoscopic index and a biomarker. The interrater reliability between a radiology resident and an expert was excellent.
OBJECTIVE: The purpose of this study was to evaluate four previously validated MRI activity scoring systems for diagnosis and grading of Crohn disease (CD) in the terminal ileum against an endoscopic and histopathologic reference standard.
SUBJECTS AND METHODS: Ethics approval and written informed consent were obtained. Subjects with known or suspected CD were prospectively recruited between December 2011 and August 2014. Each patient underwent MRI and ileocolonoscopy with terminal ileum biopsies. Four MRI scoring systems (Magnetic Resonance Index of Activity [MaRIA], Clermont score, London score, and Crohn disease MRI Index) and component features were applied by two observers and correlated to the Crohn disease endoscopic index of severity (CDEIS, 0-44) and histopathologic endoscopic acute inflammation score (0-6). Interobserver agreement (weighted kappa and intraclass correlation coefficient [ICC]) and diagnostic accuracy for active and ulcerating endoscopic or histopathologic disease were evaluated.
RESULTS: Ninety-eight patients (median age, 32 years old; 55 women, 43 men) were included. All four scoring systems showed good interobserver agreement (ICC = 0.70-0.78), moderate-to-strong correlation to CDEIS (r = 0.57-0.67) and weak-to-moderate correlation to endoscopic acute inflammation score (r = 0.38-0.49). Scoring systems' diagnostic accuracy for active and ulcerating endoscopic disease ranged from 73% to 78% and 71% to 76%, respectively, whereas for active histopathologic disease accuracy ranged from 65% to 72%. Between the scoring systems, no significant differences were found for both observers regarding interobserver agreement, correlation coefficients, and diagnostic accuracy.
CONCLUSION: All scoring systems were comparable in terms of interobserver agreement, correlation to the endoscopic and histopathologic reference standard, and diagnostic accuracy. The London score, MaRIA, and Clermont score have the additional benefit of having validated cutoff values for both active and ulcerating endoscopic disease.
OBJECTIVES: We aimed to explore the ability of magnetic resonance enterography (MRE) to impute the simple endoscopic score of Crohn disease (SES-CD) in children with CD, in whom failure of ileal intubation is common and may impair SES-CD calculation in clinical studies.
METHODS: This is a substudy of the prospective ImageKids study in which children with CD underwent ileocolonoscopy (scored by SES-CD) and MRE (scored on a 100 mm visual analogue scale [VAS] and by MaRIA). Mucosal healing (MH) was defined as SES-CD <3, MRE-VAS <20 mm, and/or MaRIA <7.
RESULTS: A total of 237 children (22 centers, age 11.5 ± 3.3 years), were enrolled. Ileal intubation has failed in 40 of 237 (17%). The agreement between SES-CD and MRE was 75% (k = 0.508, P < 0.001) in the ileum, and 68% to 85% in the colonic segments (k = 0.21-0.50, P < 0.001). The sensitivity and specificity of ileal MRE-VAS for MH were 91.7% (95% confidence interval 0.84-0.96) and 53.1% (95% confidence interval 0.43-0.63), respectively. The ileal MaRIA score (calculated in 33/40) was higher in the children without ileal intubation than in the others (20.5 ± 7.1 vs 15.1 ± 10.8, respectively, P = 0.0018). In 7% (16/237) of children, isolated active ileal disease would have been missed when considering SES-CD only. A multivariable model predicted the ileal SES-CD subscore from the MaRIA: SES-CDileum = 1.145 + 0.169 × MaRIAileum rounded to the nearest whole number (R = 0.17). Applying this model to the children without ileal intubation revealed that 29 of 33 (88%) had ileal disease; 8 of 29 patients (28%) with normal colonic SES-CD had imputed ileal SES-CD ≥3.
CONCLUSIONS: MRE is useful for imputing the ileal disease in pediatric clinical studies, overcoming the problem of ileal nonintubation.
The aim of this study is to evaluate the ability of magnetic resonance enterography global score (MEGS) to diagnose the activity of pediatric Crohn's disease (CD) and its correlation with endoscopic activity score.
MATERIALS AND METHODS:
70 pediatric CD patients (between the ages of 6 and 17) were enrolled who underwent ileocolonoscopy and magnetic resonance enterography (MRE) within 7 days. The simplified endoscopic activity score for Crohn's disease (SES-CD) and MEGS were acquired in the terminal ileum. Sensitivity and specificity of MEGS for detection disease activity against SES-CD was compared using the McNemar test. The correlation between MEGS and SES-CD was assessed by Spearman's rank estimation. The diagnostic accuracy of MEGS for active disease defined by SES-CD was calculated. Receiver operating characteristic curves (ROC) were constructed.
RESULTS:
Fifty-two pediatric CD patients (median age, 12 years old; 28 girls, 24 boys) were included. The incidence of upper gastrointestinal (GI) tract (23%) involvement and perianal lesions (42%) is high in pediatric Crohn's patients, and most of them suffer from internal hemorrhoids (86.5%). MEGS showed strong correlation to SES-CD (r = 0.70, P < 0.001). With endoscopic as the standard of reference, the MEGS had a high accuracy for the detection of inflammation (area under the ROC curve (AUC) of 0.89, sensitivity 0.95 and specificity 0.82) and for disease activity (AUC of 0.81, sensitivity 0.88 and specificity 0.75) in the terminal ileum.
CONCLUSION:
Pediatric Crohn's disease is unique. Our study has shown a good correlation between MEGS and endoscopy activity score with equal diagnostic efficacy. MEGS is a promising method to assess disease activity and perhaps be a valuable tool in following therapeutic changes.
