Time Of Inflammatory Bowel Disease Diagnosis Research Articles

Children and Adolescents Diagnosed With Inflammatory Bowel Disease Are at Increased Risk of Developing Diseases With a Possible Autoimmune Pathogenesis.

The development of diseases with a possible autoimmune pathogenesis is common in adults with inflammatory bowel disease (IBD). In early onset IBD, it may differ but the evidence is sparse. We aimed to investigate the risk and time span from IBD diagnosis to outcomes with different associated disorders with possible autoimmune pathogenesis. A register-based study included all Danish patients with early onset of IBD (≤18 years) between 1980 and 2021 and 50 matched references without IBD for each case. We examined the risk of type 1 and type 2 diabetes, celiac disease, thyroid disease, rheumatoid arthritis, psoriatic arthritis, and spondyloarthritis in Cox regression models. In total, 6822 patients with IBD were identified, and 337 728 matched references. The median age at the time of IBD diagnosis or index date for the matched references was 16 years (25-75 percentile: 13-18 years), and the median age at the time of an outcome or at the end of follow-up was 28.1 years (25-75 percentile: 21.5-37.0 years). According to the cumulative incidence plots psoriatic arthritis, and spondyloarthritis was diagnosed approximately 10 years after the IBD onset, and the remaining outcomes later. The adjusted hazard ratio after full follow-up was 4.72 (95% CI, 3.85-5.80) for psoriatic arthritis, 5.21 (95% CI, 4.17-6.50) for spondyloarthritis, 2.77 (95% CI, 1.92-4.00) for celiac disease, 2.15 (95% CI, 1.54-3.01) for rheumatoid arthritis, 1.69 (95% CI, 1.23-2.32) and 1.64 (95% CI, 1.21-2.21) for type 1 and type 2 diabetes, respectively. For thyroid disease, it was 1.16 (95% CI, 0.97-1.40). The risk estimates were significantly increased for all outcomes at the end of follow-up, except for thyroid disease, but according to the cumulative incidence plots, only psoriatic arthritis and spondyloarthritis occurred earlier in the IBD cohort than in the matched references.

A223 DEMOGRAPHIC AND CLINICAL CHARACTERISTICS OF PATIENTS WITH INFLAMMATORY BOWEL DISEASE AND PRIMARY SCLEROSING CHOLANGITIS AT THE OTTAWA HOSPITAL

Abstract Background Primary sclerosing cholangitis (PSC) is present in up to 8% of individuals with inflammatory bowel disease (IBD). Characteristics of this population are unique and data are limited. Aims To describe the demographic and clinical characteristics of all individuals with PSC-IBD managed at The Ottawa Hospital (TOH). Methods A chart review was performed for 132 patients with PSC-IBD followed at TOH between January 1998 and July 2023. Results Of 132 individuals with PSC-IBD identified at TOH, 26.5% had Crohn’s disease (CD), 68.2% had ulcerative colitis (UC) and 5.3% had IBD-unclassified. Median age at diagnosis were 25.3 (IQR 20.94) for IBD and 33.84 (IQR 23.98) for PSC, and 66.7% of all patients were male. More than 17% had a co-morbid autoimmune disease other than IBD or PSC. Of those with CD, roughly 15% had fibrostenotic or penetrating complications and 17% had perianal disease at the time of IBD diagnosis. Only 28% ever had a liver biopsy, whereas the majority were diagnosed with PSC by cholangiography. PSC distribution was isolated intrahepatic in 47%, isolated extra-hepatic in 5.1% and mixed intra/extra-hepatic in 38.5%. Clinically significant portal hypertension was present in 11.5% at PSC diagnosis, while 27.3% and 16.7% developed cirrhosis and liver decompensation, respectively by last follow-up. 17.4% underwent liver transplant, of which 30.4% developed PSC recurrence post-transplant. Conclusions Our study showed that the majority of PSC-IBD patients are young males with UC. Co-morbid autoimmune diseases are common while isolated extra-hepatic PSC is uncommon. Cirrhosis, liver decompensation and liver transplant are frequent occurrences in this population. PSC recurrence post-transplant is observed in more than 30% of patients. Funding Agencies None

P383 Chronic Recurrent Multifocal Osteomyelitis associated with Paediatric Inflammatory Bowel disease: A Multi-Center Retrospective Study from the Paediatric IBD Porto Group of ESPGHAN

Abstract Background Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a rare, autoinflammatory bone disorder. CRMO was linked with inflammatory bowel disease (IBD), either as an extra intestinal manifestation or as a paradoxical effect of anti-TNFa therapy. Our goal was to define the clinical features and natural history of patients carrying a dual diagnosis of CRMO and IBD Methods Medical records of pediatric patients with a dual diagnosis of IBD and CRMO were reviewed in nineteen centers from the Paediatric IBD Porto Group of ESPGHAN. Collected data included demographic characteristics, disease features, laboratory studies, bone imaging findings and outcomes of each disease Results Forty five patients (21 [47%] females) with a diagnosis of CRMO and IBD (32 [71%] with Crohn’s disease) were included. Median age at the time of dual diagnosis was was 10.2 (IQR 12-13.5) years. Patients were divided into 3 groups, based on whether CRMO developed before, during or after IBD diagnosis. In 15 patients (33%), CRMO was diagnosed ±3 months from the time of IBD diagnosis, with 8 (53%) and 2 (13%) exhibiting mild and moderate-severe IBD activity, respectively. In 20 children (44%) IBD preceded CRMO diagnosis by >3 months with a median time of 238 (85-344) weeks. At the time of dual diagnosis, 12 (60%) patients were in IBD remission and 5 (25%) exhibited moderate-severe disease activity; however, CRP and ESR were elevated (1.5 [0.4-3.4] mg/dL and 35 [21-55] mm/h, respectively) while median fecal calprotectin was 567 (68-1800) mcg/gr, including 4 patients <100 mcg/gr. 17 patients (85%) were on anti-TNFa medication at the time CRMO developed. In 10 (22%) patients CRMO preceded the diagnosis of IBD (>3 months before IBD evolution) with a median time 46 (25-248) weeks. At time of IBD presentation, CRMO was in remission in 5 patients (50%). 4 patients were diagnosed with IBD, despite the lack of any abnormal gastro-intestinal symptoms. In patients in which CRMO was diagnosed after or during IBD diagnosis, different therapeutic regimens were used, including anti-TNFa agents, methotrexate, ustekinumab, NSAID’s and corticosteroids. Two patients also received bisphosphonates. In patients in which CRMO was diagnosed after or during IBD diagnosis, CRMO remission, defined as lack of bone pain, was achieved in 22.2 (15-51.8) weeks. CRMO complications occurred in 4 patients and included vertebral collapse, length discrepancy, bone fracture and bone deformity Conclusion In the largest cohort to date, CRMO presentation was not necessarily related to clinically active intestinal inflammation and could present before, during or after IBD diagnosis. CRMO remission was achieved in all patients; Nevertheless, a small number of patients developed significant bone complications

Risk Factors of Low Bone Mineral Density in Newly Diagnosed Pediatric Inflammatory Bowel Disease.

Inflammatory bowel disease (IBD) is a chronic inflammatory disorder of the gastrointestinal tract with an increasing worldwide incidence. IBD is frequently diagnosed during childhood in the adolescent period of ongoing growth and development, and it can affect patients' linear growth, puberty, nutrition, and bone health. Therefore, its treatment and monitoring are critical to prevent secondary outcomes. However, few studies have highlighted the association between pediatric IBD and skeletal outcomes in Asian populations. We aimed to identify the prevalence and risk factors for low bone mineral density (BMD) in Korean children and adolescents with newly diagnosed IBD. Patients aged 10-18 years diagnosed with either Crohn's disease (CD) or ulcerative colitis (UC) who underwent lumbar spine bone mineral density (LSBMD) and femoral bone mineral density (FBMD) analyses via dual-energy X-ray absorptiometry at the time of IBD diagnosis were included. Low BMD was considered when the age- and sex-matched BMD Z-score was <-1.0. The LSBMD and FBMD Z-scores were correlated with clinical parameters, including general characteristics, anthropometry, and IBD-associated laboratory markers. Regression analyses were performed to identify the risk factors for low BMD. Although the general characteristics between CD (n = 42) and UC (n = 9) groups did not differ, the mean Z-scores of LSBMD and FBMD of the 51 subjects were -0.11 ± 1.24 and -0.58 ± 1.38, respectively. Furthermore, 7.8% and 18% of the study subjects had LSBMD and FBMD Z-scores < -2.0, whereas more than 50% had scores of 0--1.0. Among the clinical factors, body mass index (BMI) Z-score, duration of clinical manifestations, and serum alanine aminotransferase and selenium levels were associated with LSBMD Z-scores in the final multivariate regression analyses. Odds ratios of BMI < -2.0 standard deviation for low LSBMD and FBMD Z-scores were 31.97 and 41.45, respectively. A BMI Z-score < -0.93 was determined as the best cut-off for predicting low BMD. In newly diagnosed pediatric IBD, a substantial number of children are likely to have low BMD in prior to initial treatment while lower BMI, longer duration of clinical manifestation, and higher selenium concentration could affect initial BMD status. Routine bone health surveillance from initial IBD diagnosis throughout the treatment's completion is recommended for preventing the early development of secondary osteoporosis.

Duration of smoking cessation in patients with inflammatory bowel disease

Background and objectivesSmoking can worsen inflammatory bowel disease (IBD), although evidence regarding the duration of cessation is scarce. The objectives of the study were to determine the duration of abstinence and identify the characteristics of relapse in IBD patients. Patients and methodsUsing the local database of a nationwide registry of patients with IBD, we identified patients who were active smokers at the time of IBD diagnosis and invited them to participate in the study. Patients were asked about their smoking habit and those who were ex-smokers constituted the study cohort. We obtained clinical and smoking-related data of ex-smokers from medical records and telephone interviews. We measured nicotine dependence using the Fagerström Test for Nicotine Dependence (FTND). ResultsWe enrolled 121 IBD patients who were ex-smokers: 89 patients with Crohn's disease (CD) and 33 patients with ulcerative colitis (UC). The median age at cessation was lower in patients with CD (38 years) than in patients with UC (46 years) (p=0.002). Follow-up time was shorter in CD patients than in UC patients (114 vs. 168 months, p=0.035). No difference was found in the FTND score. Relapse was more common in CD patients (46%) than in UC patients (24%) (p=0.029), although time to first relapse was similar in both groups of patients (12 and 15 months, respectively; p=0.056). Nicotine dependence was the only independent factor associated with relapse. ConclusionsThe risk of smoking relapse was high, especially in CD patients, although their dependence level was similar to that of UC patients.

P158 Intestinal Ultrasound at IBD diagnosis predicts surgery – a Copenhagen IBD cohort study

Abstract Background The disease course of inflammatory bowel disease (IBD) is heterogeneous and unpredictable. Currently, we are unable to predict which patients will need IBD-related bowel resection in the first year after diagnosis. Intestinal Ultrasound (IUS) has been proven as a non-invasive modality for assessing disease activity in IBD. However, the evidence for IUS as a predictor of disease course is still limited. Here we present novel data on the predictive value of IUS performed at the time of IBD diagnosis. Methods Patients with new-onset IBD are currently being included in the ongoing multicentre prospective inception cohort study, the IBD Prognosis Study (May 2021 – April 2023). IUS is performed at the time of diagnosis and the patients are followed prospectively. Patients with proctitis are not assessed with IUS. The International Bowel Ultrasound Segmental Activity Score (IBUS-SAS) is calculated for the most inflamed segment with a high score indicating severe disease activity. IBUS-SAS (0-100) incorporates the bowel wall thickness (BWT), bowel wall stratification, colour Doppler signal, and inflammatory fat. Surgery was defined as an IBD-related bowel resection. Patients treated with corticosteroids for &amp;gt; 72 hours or with any IBD-treatment &amp;gt; two weeks prior to IUS were excluded from analysis. In this abstract, we report our preliminary results after including patients for 16 months. Results IBUS-SAS at diagnosis was available in 133 patients (out of 242 included). 68 patients were diagnosed with Crohn’s disease (CD) (ilieal: 22, colonic: 23, ileocolonic: 23) and 65 patients were diagnosed with ulcerative colitis (UC) or unclassified IBD (left-sided colitis: 20, extensive colitis: 45). Median follow-up was 226 days. During follow-up 10 patients underwent surgery (7.5%). These included 4 patients with UC (extensive colitis: 3, left-sided colitis: 1) and 6 patients with CD (ileal: 2, colonic: 2, ileocolonic: 2). Mean IBUS-SAS at diagnosis was 92.6 among patients who underwent surgery during follow-up vs. 48.8 for patients who did not undergo surgery (p&amp;lt;0.0001). Distribution of scores is shown in Figure 1. Mean BWT at diagnosis was 8.3 mm among patients who underwent surgery during follow-up vs. 5.0 mm for patients who did not undergo surgery (p&amp;lt;0.0001). In total, 12 patients had an IBUS-SAS &amp;gt; 90 at diagnosis and among these 7 (58.3%) were operated during follow-up. Figure 2 shows a Kaplan-Meier Curve of surgical free survival stratified by IBUS-SAS at diagnosis. Conclusion IUS activity at diagnosis of IBD seems to have the capability to predict short term risk of IBD-related surgery. Our results suggest IUS might be used at diagnosis to identify patients at high risk of IBD-related surgery.

Percepción de la necesidad de asesoramiento dietético y modificaciones alimentarias en pacientes con enfermedad inflamatoria intestinal

Background and aimIn inflammatory bowel disease (IBD), diet can be perceived as a trigger for relapses or clinical worsening, dietary modifications are frequent and not derived from professional advice. The aim of this study was to evaluate the perception of the need for dietary advice in patients with IBD, to know the dietary modifications adopted and, it's effect on IBD. MethodsAn anonymous structured questionnaire with a visual analog scale (0–10) was distributed to consecutive outpatients from our IBD unit. ResultsA total of 124 complete the questionnaire (54% ulcerative colitis, 46% Crohn's disease). Mean age was 47±12 years. Dietary advice provided in the clinic was assessed with a median score of 7 (IIC, 4.50–9.00). 40% sought external dietary advice, often during the first year after diagnosis (70%). The most frequent dietary recommendations from an external professional were: dairy free diet (29%), low fat (27%), gluten free (23%), and low fiber (21%). Dietary advice from external source was assessed with a median score of 7.50 (IIC, 5.50–9.50), improving digestive symptoms in 73% of cases. Regarding dietary modifications, 61% excluded some foods (57% permanently) and 11% fasted on their own decision. ConclusionsIBD patient show a clear need for dietary advice, especially at the time of IBD diagnosis. Early specific and in-depth dietary information would increase patient satisfaction and could prevent the adoption of unjustified exclusion diets.

Vaccination strategies for Korean patients with inflammatory bowel disease.

Patients with inflammatory bowel disease (IBD) are vulnerable to vaccine-preventable infectious diseases. Immunosuppressive drugs, which are often used to manage IBD, may increase this vulnerability and attenuate vaccine efficacy. Thus, healthcare providers should understand infectious diseases and schedule vaccinations for them to reduce the infection-related burden of patients with IBD. All patients with IBD should be assessed in terms of immunity to vaccine-preventable diseases at the time of IBD diagnosis, and be vaccinated appropriately. Vaccination is becoming more important because of the unprecedented coronavirus disease 2019 (COVID-19) global health crisis. This review focuses on recent updates to vaccination strategies for Korean patients with IBD.

P162 Prevalence and incidence of nonalcoholic fatty liver disease in Inflammatory Bowel Disease patients: risk factors for progression

Abstract Background Cross-sectional studies have assessed the prevalence of non-alcoholic fatty liver disease (NAFLD) in patients with inflammatory bowel disease (IBD). However, longitudinal studies are more suitable to capture dynamic changes. Our aim was to investigate the prevalence and progression of NAFLD in a cohort of IBD patients using validated non-invasive tools. The role of metabolic, disease-related and genetic factors as predictors of NAFLD was also assessed. Methods IBD patients without known chronic liver disease were enrolled between 2019 and 2021. NAFLD was defined as Hepatic Steatosis Index (HSI) ≥ 36 or controlled attenuation parameter (CAP) ≥288 dB/m. Demographic data, traditional risk factors for NAFLD, biochemical tests and disease features were retrospectively collected in a dedicated database at the time of IBD diagnosis (defined as baseline) and at the last follow-up visit. PNPLA3 rs738409 C&amp;gt;G and TM6F2 E167K C&amp;gt;T polymorphisms were also investigated. Univariate and multivariate logistic regression analysis were performed to identify risk factors for NAFLD at baseline and at the end of follow-up. Results 227 consecutive patients (mean age 46.1 ± 13.4 years, 48.7% males, 57% Crohn’s disease, mean disease duration 9.4 ±7.5 years) were enrolled. Laboratory data at the time of diagnosis were available for 64 patients. The mean HSI was 30.9 ± 4.9 and 8/64 eligible patients (12.5%) had NAFLD at baseline. Male sex (odds ratio [OR]: 5.6; 95% confidence interval [CI] 3.1–8.9; P = 0.02) and body mass index (BMI) (OR: 1.4, 95% CI 1.1–2.9; P = 0.0008) were significantly associated with NAFLD at baseline. 11/64 (17.1%) patients developed NAFLD, accounting for an incidence rate of 4.0/100 PY; this was predicted only by changes in BMI over time (OR: 13.6, 95% CI 4.8–19.5; P = 0.001). At the last follow-up visit, NAFLD was diagnosed in 31.2% (71/227) according to HSI and in 21.1% (48/227) according to CAP. Among 8 patients with NAFLD at baseline, HSI worsened in 6 patients (41.0 ± 3.3 vs 44.0 ± 5.2), while two patients had steatosis regression. At the end of follow-up, age (P = 0.01), BMI (P = 0.002) and previous surgery (P = 0.01) were independently associated with NAFLD. Conclusion Our study confirms that NAFLD is common in patients with IBD. Variables related to IBD were not associated with incident NAFLD. Age, BMI and previous surgery were independently associated with NAFLD progression, suggesting a pathogenetic role of gut microbiota and impairment in bile acids metabolism. Our results suggest the need for active surveillance for NAFLD in patients with IBD.

DOP10 Intestinal Ultrasound at IBD diagnosis predicts major disease events – A Copenhagen IBD cohort study

Abstract Background The disease course of inflammatory bowel disease (IBD) is heterogeneous and highly unpredictable. Intestinal Ultrasound (IUS) is a non-invasive modality capable of assessing disease activity in IBD. IUS is reliable, patient-friendly, and allows frequent monitoring of disease activity. However, the evidence for IUS as a predictor of disease course is still limited. Here we present novel data on the predictive value of IUS performed at the time of IBD diagnosis. Methods Patients with new-onset IBD are currently being included in the ongoing multicentre prospective inception cohort study, the IBD Prognosis Study. During the first five years following diagnosis, patients undergo regular clinical, biochemical, endoscopic, and imaging assessments. IBD treatment and disease events are prospectively recorded. IUS is performed at diagnosis, after three months, and hereafter annually. Patients with proctitis do not undergo assessment with IUS. The newly developed IUS score, the International Bowel Ultrasound Segmental Activity Score (IBUS-SAS), is calculated for the most inflamed segment, with a high score indicating severe disease activity. The score incorporates bowel wall thickness (BWT), bowel wall stratification, colour Doppler signal, and inflammatory fat. In this abstract, we report our preliminary results after including patients for six months. Results IBUS-SAS at diagnosis was available in 60 patients. 32 patients were diagnosed with Crohn’s disease (ilieal: 7, colonic: 19, ileocolonic: 6) and 28 patients were diagnosed with ulcerative colitis (UC) or unclassified IBD (proctitis: 1, left-sided colitis: 8, extensive colitis: 19). The mean IBUS-SAS at diagnosis was 51.1, with a mean BWT of 5.2 mm. Major clinical outcomes were initiation of biologic therapy, n=12 (20.0%), IBD-related bowel resection, n=5 (8.3%), IBD-related hospitalisation, n=19 (31.7%). The mean IBUS-SAS at diagnosis was 66.2 among patients with the combined endpoint of any of these disease outcomes during follow-up vs. 34.7 for no major outcomes (p&amp;lt;0.001), see Figure 1. Additionally, we found that all patients with an IBUS-SAS above 80 at diagnosis had been hospitalised and started on systemic steroids. So far, 20 patients had an IUS follow-up scan after three months showing a mean IBUS-SAS reduction by 17.0 points (p=0.008). Conclusion We present data on the predictive value of early IUS in new-onset IBD. IUS activity at diagnosis of IBD seems to have the capability to predict short term disease outcome. At diagnosis, high IBUS-SAS is associated with major disease events such as starting biological therapy, IBD-related bowel resection, and IBD-related hospitalisation. Furthermore, response to treatment is reflected by a decrease in IBUS-SAS after three months.

Growth Failure in Pediatric Inflammatory Bowel Disease: A Preliminary Analysis

Background: Inflammatory bowel disease (IBD) is a chronic relapsing disease, characterized by chronic inflammation of the gastrointestinal tract (GIT). The incidence of IBD has been increasing over the last two decades, for example, from 1999- 2008 incidence increased from 21.3 to 26.2 per 100,000. Inflammatory bowel disease is mainly classified into two types: Crohn’s disease (CD), and ulcerative colitis (UC). Both types can be identified and distinguished through laboratory tests such as ESR, CRP, and fecal calprotectin, radiographic imaging, and direct visualization via colonoscopy. The etiology of growth failure in IBD, BMI z-score of less than -2.5, is poorly understood; however, chronic inflammation, low-calorie intake, and consequential steroid treatment are the most important factors. This study aims to determine the incidence of growth failure and associated factors in pediatric patients with IBD.&#x0D; Methods: This was a retrospective cohort study of children aged 15 years and below, with the diagnosis of IBD. Data were obtained from the pediatric gastroenterology department pediatric inflammatory bowel disease database from 2007 to 2017. Our study identifies the prevalence of growth failure via a calculated BMI z-score of less than -2.5 among the pediatric inflammatory bowel disease (PIBD) population. The Chi-square test is used for categorical data and Wilcoxon rank-sum test for continuous data. A p-value &lt;0.05 was considered statistically significant. All analyses were carried out with Stata IC/15.1 (StataCorp LP, College Station, TX, USA).&#x0D; Results: A total of 36 patients were included. The mean age of children with growth failure at the time of IBD diagnosis was 8 (±4), and more than half of them (58.3%) were boys. The incidence of growth failure at the time of diagnosis was 22.2% and declined to 11.1% over 26 months. Furthermore, the mean BMI z-score gradually improved from -0.741 to -0.1 over the same period of follow-up after proper management and multidisciplinary involvement.&#x0D; Conclusion: Growth failure in IBD patients according to our data is uncommon, however, it was reduced by 50% over 26 months. This could be attributed to nutritional status improvement over the management period. We recommend following the stepwise approach according to the severity of the disease and managing IBD patients by a multidisciplinary team. However, due to the relatively small sample size and low growth failure incidence, a large-scale study is recommended to determine any causal relations.

Epstein-Barr Virus Prevalence at Diagnosis and Seroconversion during Follow-Up in Pediatric Inflammatory Bowel Disease.

Primary Epstein–Barr virus infection in pediatric patients with inflammatory bowel disease during immunomodulation with thiopurines has been associated with increased risk for malignancies or hemophagocytic lymphohistiocytosis. We determined Epstein–Barr virus (EBV) seroprevalence at inflammatory bowel disease (IBD) diagnosis and seroconversion during follow-up in a large single center cohort of children with IBD. EBV serology results and patient characteristics were retrospectively retrieved from the hospital documentation system. EBV seronegative patients at IBD diagnosis were prospectively retested. We report on IBD patients with symptomatic active EBV infection and a complicated disease course, and those diagnosed with malignancy with respect to EBV status and drug exposure. Of 402 patients, 194 (48%) had available EBV serology results at time of IBD diagnosis at a median of 12 years (IQR 9–14 years). Thereof, 102 (53%) were EBV-positive. Of 92 EBV-negative patients, 66 were retested and 17% showed a seroconversion at a mean follow-up time of 4.3 years (SD 3 years). Three children treated with azathioprine experienced acute clinically relevant EBV infection 2, 2.5, and 4 years after IBD diagnosis, two developed signs of hemophagocytic lymphohistiocytosis. Three cases of malignancy occurred in the cohort, though none seemed to be triggered by EBV. In conclusion, almost 50% of pediatric IBD patients were EBV-naïve following diagnosis and may be at increased risk to develop severe EBV infection during immunosuppressive therapy, potentially associated with complications such as hemophagocytic lymphohistiocytosis or malignancy.

P360 Aggressive Inflammatory Bowel Disease is associated to Hermansky-Pudlak Syndrome: Characterization of a Puerto Rican Cohort

Abstract Background Hermansky-Pudlak Syndrome (HPS), a rare autosomal recessive disorder, is the most common single-gene disorder in Puerto Rico. It is characterized by an aberrant biogenesis of lysosome-related organelles inducing oculocutaneous albinism, bleeding diathesis, and damage to other organs. Around 30% of patients with HPS develop granulomatous enterocolitis, a Crohn’s-like inflammatory bowel disease (IBD). The mutant HPS1 gene is associated with a more severe Early Onset IBD (EO-IBD). We wish to describe a group of patients with HPS-associated IBD. Methods Puerto Ricans with HPS and concomitant IBD were recruited in our IBD clinic for a prospective cohort study. After informed consent, demographic and medical data were collected from the patient and the medical record. Variables included sex, HPS genotype, age at the time of IBD diagnosis, IBD location and behavior according to Montreal and Paris Classification, surgical interventions, medications, and extra-intestinal manifestations (EIM). Descriptive analysis was performed calculating mean and standard deviations. This study is approved by the MSC IRB. Results 13 patients with HPS and IBD, 9 with confirmed genotype 1, were identified: 8 males and 5 females. The mean age at the time of IBD diagnosis was 22 ± 11 years. 6 were diagnosed at ≤ 16 y/o (A1), 6 were diagnosed between 17-40 years (A2) and 1 patient was in the range of EO-IBD (7 y/o). 9 patients had advanced penetrating behavior (B3) of which 7 had concomitant perianal involvement. Most patients (9) had a surgical intervention; 8 have an ostomy. The majority (10) are using biologics (4 with concurrent immunomodulators). The most frequent EIM was peripheral arthropathy seen in 45%. Conclusion HPS patients had a severe course of IBD, as shown by age at diagnosis, phenotype, the prevalence of perianal disease, surgery and use of biologics. In comparison to a database of 836 Puerto Ricans with non HPS Crohn’s Disease, those with HPS were more likely to have surgeries (69% vs 55%, P=.303) and use biologics (77% vs 43%, P=.015). Gastrointestinal symptoms including bleeding may be overlooked by the primary physician. Proactive vigilance in patients with HPS to make an early diagnosis of IBD and aggressive medical treatment may result in better outcomes for these patients.

HEPATITIS VACCINATION AND ANTIBODY STATUS AMONG U.S. ADULTS WITH INFLAMMATORY BOWEL DISEASE

Abstract Background It is difficult to predict when inflammatory bowel disease (IBD) patients will need to receive immunosuppressive therapy. The use of immunosuppressants increases the risks of opportunistic infection and re-activation of viral replication. Hepatitis A (HAV) and B (HBV) are still endemic in certain areas of the world, and the latter has the potential to induce fulminant hepatitis with hepatic failure in the immunocompromised. Thus, it is important to routinely screen IBD patients for hepatitis and recommend appropriate vaccinations for those who are seronegative for antibodies. Methods We performed a retrospective analysis with data from the National Health and Nutrition Survey (NHANES) from 2009 to 2010. We identified participants who reported whether they were told by a doctor or other health professional that they have IBD. We excluded those who did not provide pertinent information and those who did not know their vaccination status. For the purpose of statistical analysis, vaccination status was stratified as either complete or incomplete. Complete vaccination for HAV was defined as at least 2 doses, and that for HBV was defined as 3 doses. Incomplete vaccination was defined as the aggregate of inadequate doses, no doses, and refusal. Results HAV vaccination data was available in 48 IBD and 4303 non-IBD participants. Complete HAV vaccination was 14.6% among IBD patients, compared to 26.4% for non-IBD participants (odds ratio [OR] 0.47; 95% confidence interval [CI], 0.21–1.06). There were 55 IBD and 4443 non-IBD participants with known anti-HAV antibody status, and seropositivity was similar for both groups (41.8% vs. 42.4%, p=0.93). HBV vaccination data was available in 50 IBD and 4349 non-IBD participants. Complete HBV vaccination was 26.0% among IBD participants, compared to 34.4% among non-IBD participants (OR 0.67; 95% CI, 0.35–1.26). The presence of anti-HBV surface antibody was reported in 58 IBD and 4666 non-IBD participants, and seropositivity in the IBD group was less than the non-IBD group (17.2% vs. 23.5%, p=0.26). Conclusion Patients with IBD have lower rates of hepatitis vaccination and lower seropositivity for hepatitis antibodies. The current American College of Gastroenterology guidelines recommend the assessment and completion of HBV vaccination schedule prior to initiating biologic therapy. The European Crohn’s and Colitis Organisation is stricter on the timing of these preemptive measures and recommends doing so at the time of IBD diagnosis. A small number of studies have suggested that seroconversion following vaccination may be reduced in IBD patients, but even so, the vaccination rates need to be improved greatly. To do so, gastroenterologists and primary care physicians should systematically educate IBD patients on the importance of hepatitis vaccination and recommend routine testing for seronegative patients.

The Distribution of Gastrointestinal Pathogens on Stool PCR Prior to the Development of IBD.

We investigated the distribution of pathogens on stool gastrointestinal (GI) polymerase chain reaction (PCR) testing in those who subsequently developed inflammatory bowel disease (IBD). Infectious gastroenteritis has been associated with later development of IBD. This retrospective study includes patients of all ages hospitalized for diarrhea with positive GIPCR panel and subsequently a new diagnosis of IBD [confirmed by chart review and International Classification of Disease, Clinical Modification code for Crohn's disease (CD) or ulcerative colitis (UC)], between March 2015 to September 2019 at our quaternary care institution. Patients with IBD diagnosis before GIPCR were excluded. Descriptive statistics characterized the distribution of microbial pathogens in relation to later IBD diagnosis. Fifty-four participants were eligible (UC 44%; CD 56%). Median age at time of IBD diagnosis was 35 years [interquartile range (IQR) 18 to 65]. Median time between GIPCR and IBD diagnosis was 3 months (IQR 2 to 9) for all patients. When stratified by organism class, median time to diagnosis was 6 months (IQR 2 to 10) for patients with bacteria, 3 months (IQR 1 to 8) for patients with viruses, and 1 month (IQR 0.75 to 1) for patients with parasites (log-rank P=0.001). Sixty-nine unique pathogens (83% bacteria) were identified on all tests. Escherichia coli was the most common species (71%), of which enteropathogenic E. coli was predominant (38%). The E. coli species, specifically enteropathogenic E. coli, may be implicated in the development of IBD. This is one of the first studies to evaluate the results of stool GIPCR in the link between the microbiome and IBD pathogenesis.

Clinical and Laboratory Characteristics Are Associated With Biologic Therapy Use in Pediatric Inflammatory Bowel Disease: A Retrospective Cohort Study.

BackgroundBiologic agents are a highly useful class of medications for treating inflammatory bowel disease (IBD). Limited evidence exists to guide initiation of biologic therapy, especially in pediatric patients. It is unclear if disease severity is connected to biologic response. We hypothesized that the clinical, biochemical and radiographic characteristics of pediatric IBD at diagnosis were associated with subsequent initiation of biologic therapy.MethodsWe performed a retrospective analysis of the charts of all pediatric patients diagnosed with IBD at our centre over 14 years. Kaplan–Meier curves evaluated patient characteristics at diagnosis with time to initiation of biologic therapy. A Cox proportional hazards model was used for multivariate characteristic analysis.ResultsA total of 198 patients were included, 57.6% had Crohn’s disease, 27.8% had ulcerative colitis and 14.6% had IBD type unclassified. Mean follow-up time was 47.8 months. About 55.5% of the patients received a biologic medication, the mean time to biologic initiation was 21.5 months. Earlier initiation of biologic therapy was frequently associated with older age, higher disease activity index and lower serum albumin.ConclusionsOlder pediatric patients with more severely active disease and lower serum albumin levels at the time of IBD diagnosis were more likely to initiate biologic therapy when considering biologic initiation, even many years after diagnosis. Identification of these characteristics may help inform decisions to initiate biologic therapy earlier in the IBD disease course.

S3215 Hermansky-Pudlak-Associated IBD: Is It the Same? Description of a Puerto Rican Cohort

INTRODUCTION: Hermansky-Pudlak Syndrome (HPS) is the most common single gene disorder in Puerto Rico. It is a rare autosomal recessive disorder due to aberrant biogenesis of lysosome-related organelles leading to oculocutaneous albinism, bleeding diathesis, and involvement in other organs. About 30% of patients with HPS develop a form of Crohn’s-like inflammatory bowel disease (IBD), granulomatous enterocolitis. Whether this is a manifestation of HPS or true IBD is still in debate. We wish to describe a group of patients with HPS-associated IBD. METHODS: This is a prospective cohort study in which phenotypically identified patients with HPS and concomitant IBD are recruited. After informed consent, a questionnaire with demographic and medical data is administered through patient interview and record review is performed. The variables collected are sex, HPS genotype, age at the time of IBD diagnosis, comorbidities, IBD location and behavior according to Montreal Classification, surgical interventions, medications, and extra-intestinal manifestations (EIM). This study is approved by the IRB. RESULTS: Ten patients with HPS and concomitant IBD were identified in our clinics - 5 males and 5 females. HPS genotype in 9 showed 8 were HPS-1 and 1 was HPS-3. The mean age at the time of IBD diagnosis was 22 ± 10 years. Six were diagnosed between ages 17 and 40 years and 4 patients were diagnosed at ≤16 y/o. Seven patients had colonic involvement, 3 had ileocolonic involvement. Sixty percent of patients had an advanced penetrating behavior and 50% had perianal involvement. Six patients had some type of surgical intervention. Three patients were on both a biologic and an immunomodulator (IMM), 5 were on a biologic alone, 1 on IMM alone, and 1 without maintenance treatment. The most frequent EIM was anemia in 40% of patients. CONCLUSION: HPS patients had a severe course of IBD, as shown by phenotype, surgery, and use of biologics. A younger age at diagnosis and a higher than usual prevalence of perianal disease also predict a complicated course. Further studies are necessary to determine if HPS-associated IBD is related to the genetic defect and if this may result in a different clinical course than idiopathic IBD.

Obesity Is More Common in Children Newly Diagnosed With Ulcerative Colitis as Compared to Those With Crohn Disease.

This prospective observational study aimed to examine the prevalence of obesity in a population-based cohort of children and young adults newly diagnosed with inflammatory bowel disease (IBD) and assess their outcome in comparison to newly diagnosed normal/underweight patients. Our longitudinal population-based cohort comprised all children younger than 17 years diagnosed with IBD in the province of Manitoba, Canada between 2012 and 2018. Cox regression model with adjustment for a priori covariates was used to examine the time to first relapse among patients who were obese/overweight at the time of IBD diagnosis in comparison to patients who were normal or underweight. A total of 139 patients with IBD were followed up for a median duration of 1.09 (interquartile range: 0.53-2.62) years. Obesity was more common in children newly diagnosed with ulcerative colitis (UC) compared to those with Crohn disease (CD) (12.7% vs 0.0%; P = 0.005). Age at diagnosis poorly correlated with the body mass index z score (R = 0.23; P = 0.01). The proportions of patients who were underweight at the time of IBD diagnosis among patients with UC and CD were 6.3% and 20.0%, respectively (P = 0.01). The time to initial relapse was not found to be significantly associated with weight category at diagnosis in UC (adjusted hazard ratio = 0.77; 95% confidence interval: 0.40-1.63) or CD (adjusted hazard ratio = 0.83; 95% confidence interval: 0.20-3.51). Obesity was more common in children and young adults newly diagnosed with UC. The majority of the underweight children had CD.

Errors in the care of inflammatory bowel disease patients: “Errata” Study

Abstract Introduction Errors are very common in daily clinical practice; however, they can be prevented. Our aim was to identify the most common errors in the outpatient management of inflammatory bowel disease (IBD) patients. Material and methods Patients diagnosed with IBD, being treated at our IBD Unit and who were referred for a second opinion were consecutively enrolled. Data on the strategies implemented by their previous physicians were obtained. These strategies were compared with the currently recommended diagnostic and therapeutic procedures. Results Seventy-four IBD patients were enrolled. Prior to care in our Unit, screening for tobacco use had been performed in 50% of Crohn's disease patients, while smoking cessation counselling had been provided in 29%. At the time of IBD diagnosis, the hepatitis B virus immunisation status had been investigated in 16% of the patients, the hepatitis C virus status in 15%, and the varicella status in 7%. Seven percent of the patients had been vaccinated against hepatitis B virus, and 3% against influenza, tetanus and pneumococcus. Sixty-seven percent of the patients with an indication for use of 5-aminosalicylic acid and 37% of those with an indication for immunosuppressants had received the indicated drug. Discussion Errors in the outpatient management of IBD patients are very common and relevant.

Errores en la atención de los pacientes con enfermedad inflamatoria intestinal: estudio «ERRATA»

IntroductionErrors are very common in daily clinical practice; however, they can be prevented. Our aim was to identify the most common errors in the outpatient management of inflammatory bowel disease (IBD) patients. Material and methodsPatients diagnosed with IBD, being treated at our IBD Unit and who were referred for a second opinion were consecutively enrolled. Data on the strategies implemented by their previous physicians were obtained. These strategies were compared with the currently recommended diagnostic and therapeutic procedures. ResultsSeventy-four IBD patients were enrolled. Prior to care in our Unit, screening for tobacco use had been performed in 50% of Crohn's disease patients, while smoking cessation counselling had been provided in 29%. At the time of IBD diagnosis, the hepatitis B virus immunization status had been investigated in 16% of the patients, the hepatitis C virus status in 15%, and the varicella status in 7%. Seven percent of the patients had been vaccinated against hepatitis B virus, and 3% against influenza, tetanus and pneumococcus. Sixty-seven percent of the patients with an indication for use of 5-aminosalicylic acid and 37% of those with an indication for immunosuppressants had received the indicated drug. DiscussionErrors in the outpatient management of IBD patients are very common and relevant.