Faculty Bibliography

INTRODUCTION: Cytomegalovirus (CMV) primarily affects immunocompromised patients, and has multiple manifestations in the gastrointestinal (GI) tract. The incidence of CMV-related GI disease has decreased since the advent of antiretroviral therapy (ART) for HIV. This, along with varied and nonspecific symptoms, makes it difficult to diagnose. CASE DESCRIPTION/METHODS: We report a 34-year-old man with AIDS (CD4 count 114 cells/muL) on ART presenting with five days of fevers. He was admitted to the ICU for sepsis and stabilized. His course was complicated by pulmonary embolism, recurrent fevers, and development of biopsy proven pyoderma gangrenosum. He also reported odynophagia, and was empirically treated for candida esophagitis with fluconazole. On hospital day 37, he developed massive hematochezia. An urgent esophagogastroduodenoscopy (EGD) did not reveal the source of bleeding, but showed a 5-cm linear esophageal scar, which appeared to be a healing tear. Sigmoidoscopy revealed a solitary rectal ulcer with a visible vessel, which was clipped for hemostasis. His bleeding resolved. The patient's odynophagia persisted, significantly limiting his ability to tolerate oral intake. Repeat EGD revealed a large, cratered, non-bleeding esophageal ulcer which was biopsied. The same day, the patient developed hematochezia; repeat sigmoidoscopy showed a deeply cratered rectal ulcer with a visible vessel successfully treated with hemostatic clips. Esophageal biopsies returned positive for CMV; valganciclovir was initiated. His odynophagia improved after starting treatment and he had no further GI bleeding. Given the clinical response, the rectal ulcers were presumed to be due to CMV, making this a case of extensive CMV-related GI disease. DISCUSSION: This is a case of extensive CMV-related GI disease which eluded diagnosis during a prolonged and complicated hospitalization. CMV-related GI disease has varied presentations, most commonly affecting the esophagus, but has also been noted to manifest with gastritis, duodenitis, pancreatitis, and colitis. The most common endoscopic findings for esophageal involvement are discrete, shallow, punched-out ulcers surrounded by normal appearing mucosa. Clinical manifestations of colonic CMV include abdominal pain and diarrhea, while discrete ulceration causing bleeding is less commonly reported. This case highlights the importance of a high index of suspicion for CMV in immunocompromised patients with multiple, unexplained GI symptoms to avoid a delay in diagnosis and management. (Figure Presented)

INTRODUCTION: Familial dysautonomia (FD) is a progressive neurogenetic disease with carrier rate as high as 1 in 18 persons in European Jews of Polish origin. Clinical hallmarks include cardiovascular instability, spinal deformities, renal dysfunction, alacrima, ataxia, and impaired nociception. Physical or emotional stress may elicit autonomic crises characterized by hypertension and vomiting. Despite profound sensory deficits, GI perturbations are frequently reported by FD patients. While the incidence of inflammatory bowel disease (IBD) and FD is unknown, concurrence is underreported given increased frequency of both diseases in Ashkenazi Jews. CASE DESCRIPTION/METHODS: We report a 33-year-old female with FD and ulcerative colitis who presented with one week of abdominal pain and bloody diarrhea. She had been maintained on balsalazide. Colonoscopy one year prior revealed endoscopic and histologic remission. On physical examination, her abdomen was tender in the lower quadrants. A CT scan revealed pancolitis. Stool studies resulted negative. Her CRP was 58.4 mg/L and albumin was 2.4 g/dL. A flexible sigmoidoscopy noted Mayo endoscopic score 3 in the rectum and CMV staining was negative. The patient was started on IV steroids. Her hospital course was complicated by ileus, parainfluenza infection, and MSSA bacteremia with a pacemaker lead vegetation, requiring extraction. Lack of optimal clinical response to treatment on hospital day five led to consideration of alternative treatments with careful attention to her underlying FD. A subtotal colectomy with end ileostomy was unfavorable due to concern for volume loss. Infliximab and cyclosporine were opposed due to infection risk and later exhibiting possible nephrotoxicity. During this discussion the patient improved enough to be transitioned to oral steroids with a plan to initiate vedolizumab as an outpatient. On recent colonoscopy she had achieved mucosal healing. DISCUSSION: This is the first case of UC in a FD patient reported. Given myriad GI symptoms in the later diagnosis it can be hard to distinguish disease-related from treatment-related events. Due to the gut-specificity of vedolizumab, infection risk is considerably reduced compared to that of other biologics and is the most favorable option in the setting of underlying FD. This case highlights the difficulty encountered when treating IBD in the setting of systemic illness and underscores the need to carefully consider management options to enhance patient outcomes. (Figure Presented)

Education in inflammatory bowel disease (IBD) varies widely between categorical gastroenterology (GI) programs and is largely related to the presence of expert clinicians, patient population, and the presence of an IBD center. The treatment of IBD is becoming increasingly complex at a rapid pace, widening this educational divide. This manuscript outlines all the current US educational offerings in IBD for GI fellows, including how to obtain supplemental education during the 3-year training period and beyond. It reviews how to assess trainee knowledge in the field of IBD and proposes 8 clinically anchored, entrustable professional activities that should help prioritize important aspects of IBD management to incorporate during categorical GI training.

BACKGROUND: According to the 2017 American College of Gastroenterology (AGA) guidelines, therapeutic drug monitoring (TDM) of drug trough concentrations and anti-drug antibodies is recommended to optimize treatment with anti-tumor necrosis factor (TNF) agents and thiopurines1. Specifically, the AGA conditionally recommends reactive TDM in patients with active symptoms of inflammatory bowel disease (IBD) while on anti-TNF agents, as such testing is crucial for differentiating between mechanistic, non-immune mediated pharmacokinetic and immune-mediated pharmacokinetic drug failure, and allows providers to appropriately tailor treatment regimens. As such algorithms for monitoring therapies in IBD have evolved, it has become incumbent on physicians caring for these patients to develop techniques to engage in patient-centered care using the technique of shared decision making. Gastroenterology (GI) trainees may not be well versed in navigating these complex interpersonal skills. The Observed Structured Clinical Examination (OSCE) is a well-validated method of assessing core competencies of communication and professionalism. While it is traditionally used at the undergraduate medical education level, it has been used at the graduate level to address disease-specific competencies. Here, we discuss the use of an OSCE to assess the performance of GI fellows in engaging in shared decision-making on the topic of TDM for a patient with complex IBD refractory to treatment.
METHOD(S): Eleven second-year gastroenterology fellows from 4 GI fellowship programs participated in a 4 station OSCE. Previously validated OSCE checklists were used to assess the fellows' performance in IBD-specific cases, one of which will be discussed here. In the "Therapeutic Drug Monitoring" case, the objective for the fellows was to discuss the indications for infliximab trough and antibody testing and how results of this testing would impact treatment based on the AGA guidelines. Checklists were scored on a 3 and 5-point Likert Scale by the Standardized Patient (SP), mapped to the appropriate ACGME milestones by a GI medical educator and normalized on a scale from 0 to 9. Post-OSCE, the fellows were surveyed to assess their perspective on their performance as well as the exam's educational value.
RESULT(S): 6 ACGME milestones were assessed in this OSCE. Scores ranged from mean of 5.85 to 7.88. Fellows scored lowest on gathering and synthesizing essential and accurate information to define each patient's clinical problem(s) (PC1, mean score 5.85) with an average score of 5.85. They scored highest for overall clinical knowledge (MK1, mean score 7.88). Overall, 9/10 (90%) of fellows would be recommended for their interpersonal skills, but only 4/10 (40%) were deemed effective in their communication skills. The majority of fellows noted improvement in their understanding of when to use and how to interpret TDM after the exercise. CONCLUSION(S): This OSCE was designed to assess clinical and communication skills for gastroenterology surrounding the complex clinical arena of therapeutic drug monitoring utilizing crucial communication skills. The results suggest weaker performance linked to gathering and synthesizing clinical information, with stronger performance in clinical knowledge, developing management plans and various communication skills. This OSCE feedback and assessment can be used to develop targeted educational interventions to strengthen clinical and communication skills for providers

BACKGROUND: Infliximab and ustekinumab are used to treat Crohn's disease (CD). Neurologic side effects have rarely been described with either agent.We report a case of a 75-year-old female with fibromyalgia and inflammatory bowel disease with subsequent development of sporadic Creutzfeldt- Jakob disease (sCJD) after initiation of biologic therapy. CASE: The patient was diagnosed with ulcerative colitis (UC) in 1992 and treated with 5-ASA, 6- MP, and steroids. She stopped medication shortly thereafter but symptoms returned in November 2016 requiring hospitalization. She was treated with IV steroids, but transitioned to infliximab due to suboptimal response. In February 2017, she exhibited flushing, dizziness, and muscle spasms after an infusion. Infliximab antibody levels were >'100 U/mL. Prior to transitioning therapies, a colonoscopy was performed and noted improved colitis, but biopsies showed granulomas and her diagnosis was changed to CD. A CT enterography revealed disease limited to the colon. A plan to treat the patient with ustekinumab was made in July 2017. Of note, during several office visits, the patient demonstrated hand tremor, imbalance, and widened gait she believed to have started around the time of her adverse reaction to infliximab in February 2017. The symptoms were initially thought to be related to steroid exposure, however tapering of steroids did not lead to improvement. Despite many attempts, the patient declined neurological evaluation. The symptoms worsened throughout the duration of ustekinumab therapy, and in February 2018, the patient sustained a left distal radius fracture after a fall. She agreed to a neurological evaluation in May 2018 and was found to have several focal cerebellar deficits. EEG was nonspecific. Brain MRI was notable for symmetrically increased signal within the caudate nucleus, putamen, and thalami, and prominent ventricles compatible with cerebral and cerebellar volume loss-findings suggestive of sCJD. A lumbar puncture revealed normal 14-3-3 protein in the CSF, but real-time quaking induced conversion was found to be positive. Following a goals of care discussion the patient was transitioned to home hospice. DISCUSSION: Trials demonstrating the efficacy of infliximab report an excellent safety profile, with rare reports of neurologic side effects. These include optic neuritis, confusion, paresthesias, and gait instability thought to be due to TNF-a blockade leading to extensive demyelination throughout the central and peripheral nervous system. Trials demonstrating the efficacy of ustekinumab also report a positive safety profile with rare neurologic side effects. We found 4 case reports of severe neurologic diseases including one case of primary progressive multiple sclerosis, amyotrophic lateral sclerosis, and 2 cases of reversible posterior leukoencephalopathy syndrome throughout treatment. These occurred in patients receiving treatment doses for either psoriasis or CD. To our knowledge, there have been no preceding case reports of sCJD development following either infliximab or ustekinumab. With respect to the onset of sCJD in our reported case, the temporal onset of symptoms succeeding the patient's infusion reaction to infliximab and commencement of ustekinumab therapy does raise the possibility of an association, however it may be a mere coincidence. Further research into the possible long-term neurologic effects of infliximab and ustekinumab is warranted

Background/UNASSIGNED:Clostridium difficile infection (CDI) is common in patients with inflammatory bowel disease (IBD), often leading to diagnostic confusion and delays in IBD therapy escalation. This study sought to assess outcomes after CDI in IBD patients exposed to new or escalated immunosuppressive therapy. Methods/UNASSIGNED:This multicenter retrospective cohort study included IBD patients with documented CDI at 4 academic medical centers. Data were abstracted from clinical databases at each institution. Outcomes at 30 and 90 days were compared between patients undergoing new or intensified immunosuppressive therapy and those without therapy escalation. Continuous variables were compared using t tests, and proportions using chi-square tests. Multivariable logistic regression was used to determine the association of individual variables with severe outcomes (including death, sepsis, and/or colectomy) within 90 days. Secondary outcomes included CDI recurrence, rehospitalization, worsening of IBD, and severe outcomes within 30 days. Results/UNASSIGNED:A total of 207 adult patients with IBD and CDI were included, of whom 62 underwent escalation to biologic or corticosteroid therapy (median time to escalation, 13 days). Severe outcomes within 90 days occurred in 21 (15.6%) nonescalated and 1 (1.8%) therapy-escalated patients. Serum albumin <2.5 mg/dL, lactate >2.2 mg/dL, intensive care unit admission, hypotension, and comorbid disease were associated with severe outcomes. Likelihood of severe outcomes was decreased in patients undergoing escalation of IBD therapy after CDI (adjusted odds ratio [aOR], 0.12) and increased among patients aged >65 years (aOR, 4.55). Conclusions/UNASSIGNED:Therapy escalation for IBD within 90 days of CDI was not associated with worse clinical outcomes. Initiation of immunosuppression for active IBD may therefore be appropriate in carefully selected patients after treatment of CDI.

Background: Patients with inflammatory bowel disease (IBD) have many unique health maintenance needs and ofen require therapy necessitating close monitoring. Gastroenterologists ofen serve as the primary care provider for these patients and therefore must be familiar with the health maintenance needs of IBD patients. In this study, we investigated whether implementing a multi-modal educational intervention could improve providers' rates of addressing healthcare maintenance measures. METHODS: A retrospective chart review was performed in 2013-2014 on 208 IBD patients to determine adherence to performance practice measures. From February-April 2016, fellows received a recurring in-service lecture and an IBD clinic note template outlining the 2011 healthcare maintenance recommendations by the American Gastroenterological Association. An iBook was also introduced, which provided a comprehensive overview of IBD practice guidelines. Retrospective chart review was then performed 1 year aferwards. For each patient, performance measures were assessed in both pre-and post-intervention notes in the following categories: vaccinations, bone health, therapy-specifc maintenance, tobacco cessation, and cancer screening. Each performance measure was given a score of 0 (not addressed), 1 (addressed), or N/A (irrelevant to subject). Te primary outcome was improvement in rates of adherence to performance measures. Te adherence rates for pre-and post-intervention groups were compared using a chi-squared test. RESULTS: A total of 208 pre-intervention clinic visits and 40 post-intervention visits were included for analysis. Afer the interventions, the rate of healthcare maintenance measures addressed overall increased from 37% to 52% (P<.001) (Figure 1). Tere were statistically signifcant improvements in addressing bone health (29% to 63%, P<.001), vaccination (33% to 47%, P<.001), and therapy-specifc measures (53% to 74%, P=.01). Tere were no statistically signifcant changes in addressing cancer screening (66% to 58%, P=.19) or smoking (23% to 30%, P=.59). CONCLUSION(S): Te use of multiple educational interventions to enhance delivery of IBD healthcare maintenance resulted in improved adherence to healthcare maintenance measures. Targeted educational programs and a multi-modal approach may be an effective method for teaching GI fellows and reinforcing the importance of addressing these measures to optimize the care of their IBD patients

Background: Te Accreditation Council for Graduate Medical Education (ACGME) has identifed six core competencies in which trainees are expected to demonstrate profciency. Milestones have been developed to provide a framework for evaluating trainee performance within these competencies. We used an objective structured clinical examination (OSCE) focused on inflammatory bowel disease (IBD) to assess the milestones in gastroenterology (GI) fellows. METHODS: Ten second-year fellows from six GI fellowship programs participated in a four case OSCE. In the "Transition of CareTM case the fellow was to assess a patient's readiness on the planned transition from child-centered to adult-centered care. In the "Shared Decision MakingTM case the fellow was to evaluate a patient with Crohn's disease who would beneft from combination therapy. In the "ER FlareTM case the fellow was to triage and suggest management of a flaring ulcerative colitis patient. In the "IBS in IBDTM case the fellow was asked to discuss irritable bowel syndrome in the context of quiescent IBD. Previously validated OSCE checklists were used to assess the GI fellows' performance using a 3-and 5-point behaviorally-anchored Likert Scale. Checklists were scored by the standardized patient. Checklist items were mapped to appropriate ACGME milestones by a GI medical educator. Scores within each milestone were normalized on a scale from 0-9 as utilized by the ACGME in the Next Accreditation System milestone initiative. Fellows were provided feedback on their performance. RESULTS: Te majority of fellows scored between 6 and 9 in the milestones assessing patient care (PC), medical knowledge (MK), interpersonal and communication skills (ICS), professionalism (Prof), and systems-based practice (SBP). Composite average scores for all participants were as follows: PC1 7. 7, PC2 6. 9, MK1 6. 9, MK2 7. 0, ICS1 7. 4, Prof1 7. 6, Prof3 6. 9, and SBP4 6. 4. Fellows scored highest in the "Shared Decision MakingTM case and scored lowest in the "Transitions of CareTM case. CONCLUSION(S): In this OSCE GI fellows performed well in the majority of milestones evaluated, however areas of less optimal performance were identifed, providing areas for future focus in fellow training. Te OSCE is a well-validated standardized tool for evaluating trainees, and with appropriate mapping of checklists to ACGME milestones, it can serve as an objective method to assess GI fellows' progress in the core competencies

Background: Nearly one-half of Crohn's disease patients require bowel resection within the frst 10 years of disease (1). Small bowel obstruction (SBO) is the most common indication for surgery in Crohn's patients, followed by abscess and presence of fstulizing disease (2). Tere are little data regarding pharmacologic treatment of Crohn's-associated SBO with corticosteroids. In particular, the safety and efcacy of corticosteroids in treating inflammation in the setting of acute Crohn's SBO remains unclear. METHODS: Our group performed a retrospective chart review of patients admitted with Crohn's-disease associated SBO to our institution. Key variables examined included use of corticosteroids, length of stay, infectious complications, and short-term requirement for surgery. Inclusion criteria included adults (>18 years) who were not pregnant and carried a known diagnosis of Crohn's disease. Using the i2b2 search engine, patients admitted with the ICD10 diagnoses for Crohn's disease and a primary diagnosis of SBO were included. Analysis of outcomes was performed comparing patients who received steroids versus those who did not using t-statistics and chi-square analysis. RESULTS: Between 2015 and 2017, ffy-seven patients met inclusion criteria. Te majority (n=32, 56%) received no corticosteroids for the preceding three months nor during the admission for SBO, while the minority (n=25, 44%) did receive steroids. Te mean age of patients (45+/-19 years vs 46+/-18 years, P=0.92), and duration of Crohn's disease (14+/-13 years vs 14+/-12 years, P=0.93) did not differ between groups. C-reactive peptide (CRP) on admission did not differ between groups (23.9+/-17 vs 46.6+/-78, P=0.49). Eleven patients (19%) required surgery related to Crohn's disease during or within the three months following admission. Tere was no difference in requirement for surgery between groups. In multivariable logistic regression, the only factor associated with requirement for surgery was duration of Crohn's disease (P<0.05). Tere was no difference in duration of nasogastric tube placement, time to PO challenge, or length of hospital stay. Tere were no mortalities in either group and no difference in infectious complications afer discharge. CONCLUSION(S): Tese results suggest that corticosteroids are not associated with improved outcomes in patients with Crohn's associated SBO. Length of stay is not decreased due to use of corticosteroids. Te study is limited by its retrospective design and small sample size. However, future case-control or randomized clinical trials can examine the use of corticosteroids during acute Crohn's-associated SBO