Endoscopic Evaluation and Management of Perianal Disease.

Perianal Crohn's disease is associated with decreased quality of life, high morbidity, and high health care use. Treatment goals are to provide infection control, stop fistula drainage, and heal fistula tracts while preserving the anorectal sphincter. Treatment includes a combination of medical and surgical intervention, but new techniques are using interventional endoscopy to act as either a bridge to surgery or to decrease the need for surgical intervention. The decision on which approach to pursue requires knowledge of prior surgery, altered anatomy, prior fistula treatment, and imaging and endoscopic evaluation of fistula complexity, mucosal involvement, and associated stricture or abscess.

Avoidant/Restrictive Food Intake Disorder Characteristics and Prevalence in Adult Celiac Disease Patients.

Background and Aims: The objective of this study was to identify the prevalence of avoidant/restrictive food intake disorder (ARFID) in patients with celiac disease (CD) and assess metabolic complications, disease control, diet adherence, and correlation with symptom and quality-of-life metrics. Methods: This was a retrospective study of 137 adult patients with CD who completed an ARFID survey in the CD clinic between 2018 and 2020. Demographics, clinical results, standardized diet assessment, and results of Celiac Disease Symptom Diary and Impact of a Gluten-free Diet Questionnaire were reviewed. The primary outcome measured was the rate of suspected ARFID based on patient-reported survey responses. Results: Seventy-eight patients (57%) met suspected ARFID criteria. There were no differences in age, gender, body mass index, micronutrient deficiencies, or bone disease in those with or without ARFID. Patients with ARFID did not have a difference in biopsy activity or better adherence to a gluten-free diet compared with non-ARFID patients. Food and social burden on Impact of a Gluten-free Diet Questionnaire was most predictive of ARFID. Conclusion: ARFID is common and has a high impact in patients with CD. Although some eating behavior is certainly due to their CD, there was no distinct difference in disease control between those with or without suspected ARFID, suggesting these maladaptive behaviors are not necessary for disease control. We did not find increased metabolic complications, but this was a 2-year snapshot. We need to further understand the social and food impacts on patients who score high on this survey to prevent further deficiencies and impaired, long-term detrimental eating behaviors.

The Importance of Monitoring the Postpartum Period in Moderate to Severe Crohn's Disease.

BACKGROUND: Prior research demonstrates Crohn's disease patients often do well in pregnancy; however, less is known about the risk of flare in the postpartum period. METHODS: A retrospective chart review was conducted at a tertiary care inflammatory bowel disease center. All pregnant women with Crohn's disease who were followed in the postpartum period, defined as 6 months after delivery, were included. Statistical analysis included χ 2 analysis, Wilcoxon rank sum test, and logistic regression analysis. The primary outcome of interest was rate of flare in the postpartum period. RESULTS: There were 105 patients included in the study, with a majority (68%) on biologic medication during pregnancy. Thirty-one patients (30%) had a postpartum flare at a median of 9 weeks (range 2-24 weeks). Twenty-five patients (81%) had their postpartum flare managed in the outpatient setting with medications (only 4 of these patients required prednisone). 6 of 31 patients (19%) were hospitalized at a median of 4 weeks (range 2-26 weeks) after delivery, requiring intravenous corticosteroids or surgery. In multivariable regression, there was no significant increase in risk of postpartum flare with increasing maternal age, flare during pregnancy, or steroid or biologic use during pregnancy. Smoking during pregnancy increased risk of postpartum flare (odds ratio, 16.2 [1.72-152.94], P < 0.05). CONCLUSION: In a cohort of Crohn's disease patients, 30% experienced a postpartum flare despite being on medical therapy, but most were able to be managed in the outpatient setting.

A Single Center Experience With Long-Term Ustekinumab Use and Reinduction in Patients With Refractory Crohn Disease.

BACKGROUND: Ustekinumab was approved for moderate and severe Crohn's disease (CD) in 2016, but little is known about long-term outcomes. METHODS: A retrospective study evaluated all patients with CD treated with ustekinumab, including patients with reinduction. C-reactive protein (CRP), Harvey-Bradshaw Index (HBI), Short Inflammatory Bowel Disease (SIBDQ), and endoscopy outcomes were collected prospectively. RESULTS: Ninety-six patients received ustekinumab, resulting in improvement in CRP, HBI, and SIBDQ scores with 68% endoscopic improvement/remission. Thirty-four patients underwent reinduction, resulting in improved HBI and CRP. CONCLUSIONS: Ustekinumab in refractory CD results in significant clinical and endoscopic improvement and reinduction may be a viable option to recapture response.

Recurrent demyelinating myelitis associated with hepatitis C viral infection.

We report a 46-year-old-man who developed recurrent myelitis associated with hepatitis C viral (HCV) infection. Spinal cord biopsy showed acute demyelination without evidence of vasculitis. Antibodies to HCV were present in the CSF; HCV RNA was not detected in the CSF. Neither HCV antigens nor RNA were detected in the spinal cord biopsy, whereas they were found in the liver biopsy. Evaluation for other infectious or autoimmune causes was unrevealing. These observations suggest that recurrent myelitis in this patient is etiologically related to HCV infection, possibly via an immune-mediated mechanism. This is the first report of pathologically proven myelitis associated with HCV infection and we suggest that HCV be considered in the differential diagnosis of the transverse myelitis syndrome.

Salivary-type neoplasms of the breast and lung.

Salivary-type tumors occur in multiple sites in the human body, likely related to a basic structural homology between exocrine glands in these different anatomic areas. This paper reviews these salivary gland tumor types in breast tissue and lung. Salivary-type tumors of both breast and lung are relatively uncommon in comparison to their salivary gland counterparts. This may be attributable in part to lack of familiarity with these tumors in extra-salivary sites, and in part to histologic overlap with other primary and metastatic tumor types. Recognition of these entities is improving as the clinical and pathologic features are better delineated, and tumors are more accurately classified. Prediction of malignant behavior is not always possible in these unusual sites. In some instances, such as adenoid cystic carcinoma, behavior is known to differ considerably from that of analogous primary salivary gland tumors and in other instances there are simply too few reported cases to allow for adequate prognostication. In fact, more recent papers discuss the need to consider a spectrum encompassing benign and malignant lesions, in both breast and lung. Of course, some entities show clear-cut evidence of malignancy with documented potential for metastasis, others show bland features and well-reported benign behavior, and the less well-defined entities reside between these two extremes. The molecular pathology of salivary gland tumors has been reasonably well investigated in that location; however; there are few molecular studies devoted to salivary-type tumors of the breast and lung. This represents a potential area for future growth in further clarifying these tumors and their behavior.

A case of vanishing bile duct syndrome and IBD secondary to Hodgkin's lymphoma.

BACKGROUND: A 39-year-old man presented with a 2-month history of abdominal pain, jaundice, non-bloody diarrhea, weakness, and weight loss. Initial evaluation revealed intrahepatic ductopenia consistent with vanishing bile duct syndrome and IBD, type unclassified. Although treatment with budesonide improved his symptoms, they worsened several months later. On repeat evaluation, he was found to have extensive lymphadenopathy and an elevated white blood cell count. INVESTIGATIONS: Physical examination, laboratory investigations, abdominal ultrasound, CT scans, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, colonoscopies with biopsies, hepatic biopsy, axillary lymph node biopsy. DIAGNOSIS: Hodgkin's lymphoma with secondary vanishing bile duct syndrome and IBD, type unclassified. MANAGEMENT: The initial symptoms were managed with budesonide, but following recurrence, the patient's underlying lymphoma was treated with nitrogen mustard and dexamethasone.