Idiopathic terminal ileitis: myth or true entity?

PURPOSE OF REVIEW: Isolated terminal ileitis is an increasing phenomenon identified during colonoscopy. Idiopathic terminal ileitis (IDTI) is a diagnosis of exclusion, representing a significant challenge from a diagnostic and management point of view. This review provides an overview of the most recent and relevant evidence on idiopathic IDTI, focusing on its evolution, the natural history and the management strategies proposed in the literature. RECENT FINDINGS: IDTI is uncommon, with a reported prevalence between 0.5 and 7%. The main differential is with Crohn's disease and intestinal tuberculosis in endemic countries. A proportion of patients (0-50%) can progress and develop Crohn's disease; however, there are no reliable predictive factors to stratify IDTI patients. SUMMARY: IDTI is a challenging entity, with a small proportion of patients progressing to Crohn's disease over time thus requiring follow-up. Noninvasive modalities such as capsule endoscopy are useful for follow-up, but further research is required to better understand this entity.

Evaluation of acute terminal ileitis in children before and during the COVID-19 pandemic.

OBJECTIVES: The aim of this study was to examine the clinical features of acute terminal ileitis in children and evaluate its rate before and during the COVID-19 pandemic. METHODS: This retrospective study was performed in our pediatric emergency department between 2018 and 2022. The records of 5363 patients who required abdominal imaging due to acute abdomen were analyzed, and 143 patients with terminal ileitis were included. The rate and etiological causes were compared during and before the COVID-19 pandemic. RESULTS: The rate of acute terminal ileitis has increased over the years. The fastest increase was in 2021, when the COVID-19 pandemic was experienced. While 59 (41.2%) patients showed acute nonspecific ileitis, the most common etiologic cause that could be identified was acute gastroenteritis. It was determined that multisystem inflammatory syndrome in children was among the causes of ileitis after the COVID-19 pandemic and was one of the top three causes. CONCLUSIONS: Acute terminal ileitis, which has many etiologies, is one of the rare radiological findings in acute abdominal pain. Examination and laboratory findings are not specific. Guidelines are needed for the investigation of the underlying etiology of acute terminal ileitis in children. The incidence of acute terminal ileitis is increasing, and the increase has been found to be faster after the COVID-19 pandemic.

[Enteritis cystica profunda]. / L'entérite kystique profonde.

Enteritis cystica profunda is a rare and benign disease defined as the invagination of the intestinal epithelium into the submucosa and more profound layers of intestinal wall leading to the formation of mucin-filled cystic spaces. We reported the case of a 45-year-old female, suffering from a Crohn's disease, with a Koenig's syndrome, diarrhea, abdominal pain and weight loss. The colonoscopy and the abdominopelvic scan showed a terminal ileal stenosis, with parietal calcifications. A surgical ileocecal resection was decided. Gross examination of the ileocecal resection showed a thickening of the ileal wall, with many mucin-filled cysts measuring 1mm to 2cm, with some calcifications. The ileal mucosa was ulcerated, and showed a stenotic sector extending over 3cm. Histological examination showed acute ulcerated ileitis lesions, with chronic ileitis lesions and stenosis, compatible with the known diagnosis of Crohn's disease. There were also many cysts into the ileal wall. They were lined with a regular ileal epithelium. The cysts contained mucus, with some calcifications. Some cysts were ruptured, with extravasation of mucus within the wall. Cystica profunda can be found anywhere along the digestive tract. The physiopathology is not yet well understood, but it seems to be favored by chronic aggression of the intestinal wall. This pathology most often coexists with Crohn's disease. The main differential diagnosis is mucinous adenocarcinoma. Cystica profunda does not require any specific treatment.

Chemotherapy-induced ileitis associated or not with colitis in digestive oncology patients: An AGEO multicentre study.

BACKGROUND: The characteristics and management of ileitis induced by chemotherapy in cancer patients are poorly described in the literature. METHODS: This retrospective multicentre study enroled patients hospitalized in a digestive oncology unit for a symptomatic chemotherapy-induced ileitis. RESULTS: Forty-three patients were included, with a regimen based on fluoropyrimidine and/or irinotecan in 95% of cases. Five patients were excluded due to the diagnosis of infectious ileitis (Clostridium difficile in 3 patients, Campylobacter jejuni in 1 patient and cytomegalovirus in 1 patient). The most frequently described symptoms were diarrhoea (77% including 54% of grade 3-4 diarrhoea), abdominal pain (58%), fever (51%) and vomiting (56%). An ileo-colonoscopy was performed in 35% of patients and did not show any specific results or severity criteria. The ileitis was complicated by bowel perforation and/or obstruction in 3 patients. Disease progression was favourable in 1-2 weeks in the vast majority of cases, on symptomatic treatment, allowing resumption of the chemotherapy regimen involved in 67% of patients. CONCLUSION: Chemotherapy-induced ileitis is a rare complication that most often involves fluoropyri-midine- and/or irinotecan-based regimens. In most cases, endoscopic examinations were not contributory and do not seem useful in the event of non-severe symptomatology which most often develops favourably on symptomatic therapy, allowing resumption of the chemotherapy involved.

Isolated Terminal Ileitis in Children.

Isolated terminal ileitis in adults is a well described entity that rarely progresses to Crohn disease (CD), and pediatric literature on this topic is very limited. We describe the prevalence, clinical, endoscopic, histologic, and radiological features, along with long-term outcome of isolated terminal ileitis in our institution. We reviewed charts of 956 children who underwent colonoscopy from 2013 to 2017. Thirty-three children had isolated histologically-defined terminal ileitis. Seventeen children were diagnosed with CD and 16 children had idiopathic terminal ileitis. Children with CD had higher prevalence of abnormal C-reactive protein levels, severe inflammation, and radiological evidence of bowel wall thickening compared with children with idiopathic ileitis. Children with idiopathic ileitis did not develop CD over a follow-up period of 83 months. In contrast to adults, CD is common in children with isolated terminal ileitis and those with idiopathic ileitis do well over long-term.

A 60-Year-Old Woman with Primary Biliary Cholangitis and Crohn's Ileitis Following the Suspension of Ursodeoxycholic Acid.

BACKGROUND There is a recognized association between inflammatory bowel disease (IBD) and hepatobiliary autoimmune disease, particularly primary sclerosing cholangitis (PSC). There have been fewer reported cases of IBD and primary biliary cholangitis (PBC), which is treated with ursodeoxycholic acid (UDCA). This report presents the case of a 60-year-old woman with PBC who was diagnosed with Crohn's ileitis after suspension of UDCA treatment. CASE REPORT A 66-year-old female patient with PBC was admitted to our department for irrepressible chronic diarrhea and recurrent abdominal pain. PBC was diagnosed on the basis of serological data: chronic (>6 months) increase in alkaline phosphatase (ALP) associated with positivity for specific anti-nuclear antibodies (sp100 and gp210), without requiring a liver biopsy and a magnetic resonance cholangiopancreatography to rule out PSC. Given the intolerance and non-responsiveness according to the Toronto criteria (ALP <1.67 times the normal limit after 2 years) to UDCA at 15 mg/kg/day, an oral monotherapy treatment using obeticholic acid at 5 mg/day was prescribed. The patient complained of abdominal pain and upper gastrointestinal symptoms. The endoscopic/histologic and radiologic examinations supported the diagnosis of Crohn's ileitis. Given the potential benefits to PBC patients of what is described as off-label therapy, budesonide at a dosage of 9 mg/day p.o. was also administered. One month after discharge, an improvement was observed both in the cholestasis indices and in gastrointestinal symptoms. CONCLUSIONS This report presents a case of PBC in which the patient was diagnosed with Crohn's ileitis after cessation of treatment with UDCA, and highlights the importance of recognizing the association between autoimmune hepatobiliary disease and IBD.

Cytomegalovirus ileitis with protein-losing enteropathy in an immunocompetent adult.

Symptomatic cytomegalovirus (CMV) infection in immunocompetent hosts has traditionally been considered to have a benign, self-limited course, and those who need intensive therapy are rare. Moreover, there are few reports of CMV infection with protein-losing enteropathy (PLE). We present an immunocompetent 74-year-old woman with CMV ileitis with PLE, which was diagnosed due to severe hypoalbuminemia and edema of the lower extremities. The patient was not immunocompromised, because a human immunodeficiency virus (HIV) antibody test was negative and she had not been taking immunosuppressants. Imaging tests including colonoscopy revealed ileitis with shallow widespread ulcers. 99mTc-human serum albumin (HAS-D) scintigraphy suggested a possibility of protein loss in the ileum based on selective accumulation of nuclides in the right abdomen. Histological findings of the biopsy showed ulcerative mucosa with abnormal cells, which had enlarged nuclei with intranuclear inclusion bodies, including typical Cowdry A type. In immunohistochemistry, these cells were positive for anti-CMV staining. She was successfully treated with medical treatments including intravenous injection of ganciclovir (GCV) (500 mg/day). We described an extremely rare case of CMV ileitis with PLE in an immunocompetent adult who was treated successfully with medical treatments, including GCV.

Prevalence and Progression of Incidental Terminal Ileitis on Non-diagnostic Colonoscopy: A Systematic Review and Meta-analysis.

BACKGROUND: Incidentally diagnosed terminal ileitis [IDTI] has been reported among asymptomatic persons undergoing non-diagnostic colonoscopy. The purpose of our study was to determine the prevalence and long-term outcomes of asymptomatic terminal ileitis. METHODS: We performed a systematic review using three biomedical databases [Medline, Embase, and Web of Science] and relevant scientific meeting abstracts. We identified observational studies that reported the prevalence of IDTI in adults undergoing screening or polyp surveillance colonoscopy and/or the long-term outcomes of such lesions. A random-effects meta-analysis was conducted to determine the pooled prevalence rate of IDTI. The progression of IDTI to overt Crohn's disease [CD] was also described. RESULTS: Of 2388 eligible studies, 1784 were screened after excluding duplicates, 84 were reviewed in full text, and 14 studies were eligible for inclusion. Seven studies reported the prevalence of IDTI in 44 398 persons undergoing non-diagnostic colonoscopy, six studies reported follow-up data, and one study reported both types of data. The pooled prevalence rate of IDTI was 1.6% (95% confidence interval [CI] 0.1-21.8%) with significant heterogeneity [I2 = 99.7]. Among patients who had undergone non-diagnostic colonoscopy and had follow-up data [range 13-84 months reported in five studies], progression to overt CD was rare. CONCLUSIONS: IDTI is not uncommon on non-diagnostic colonoscopies. Based on limited data, the rate of its progression to overt CD seems low, and watchful waiting is likely a reasonable strategy. Further long-term follow-up studies are needed to inform the natural history of incidental terminal ileitis, factors that predict progression to CD, and therapeutic implications.

Pre-pouch Ileitis is Associated with Development of Crohn's Disease-like Complications and Pouch Failure.

BACKGROUND AND AIMS: It is unclear whether pre-pouch ileitis heralds an aggressive inflammatory pouch disease in patients with ileal pouch-anal anastomosis [IPAA]. We compared outcomes of patients with pouchitis and concomitant pre-pouch ileitis with those with pouchitis alone. METHODS: Patients undergoing IPAA surgery for inflammatory bowel disease, who subsequently developed pouchitis with concomitant pre-pouch ileitis [pre-pouch ileitis group], were matched by year of IPAA surgery and preoperative diagnosis [ulcerative colitis or inflammatory bowel disease-unclassified] with patients who developed pouchitis alone [pouchitis group]. Primary outcomes were development of Crohn's disease [CD]-like complications [non-anastomotic strictures or perianal disease >6 months after ileostomy closure] and pouch failure. Secondary outcomes were need for surgical/endoscopic interventions and immunosuppressive therapy. Log-rank testing was used to compare outcome-free survival, and Cox regression was performed to identify predictors of outcomes. RESULTS: There were 66 patients in each group. CD-like complications and pouch failure developed in 36.4% and 7.6% patients in the pre-pouch ileitis group and 10.6% and 1.5% in pouchitis group, respectively. CD-like complications-free survival [log-rank p = 0.0002] and pouch failure-free survival [log-rank p = 0.046] were significantly lower in the pre-pouch ileitis group. The pre-pouch ileitis group had a higher risk of requiring surgical/endoscopic interventions [log-rank p = 0.0005] and immunosuppressive therapy [log-rank p <0.0001]. Pre-pouch ileitis was independently associated with an increased risk of CD-like complications (hazard ratio [HR] 3.8; p = 0.0007), need for surgical/endoscopic interventions [HR 4.1; p = 0.002], and immunosuppressive therapy [HR 5.0; p = 0.0002]. CONCLUSIONS: Pre-pouch ileitis is associated with a higher risk of complicated disease and pouch failure than pouchitis. It should be considered a feature of CD.

Yersinia pseudotuberculosis infection with severe localised inflammation and ulceration of the ileum in a heart transplant patient.

In this case, we present an uncommon gastrointestinal infection in an immunocompromised patient that was solely diagnosed because of close collaboration between treating physicians and microbiologists. The patient is a 42-year-old male who underwent heart transplantation 5 years earlier. He presented with fever, weight loss, diarrhoea and tiredness. Initial investigations could not elucidate the aetiology of his symptoms. The patient was referred to the department of infectious diseases for further evaluation. Serology for Yersinia species was ordered and the result was suggestive for the possibility of a Yersinia species infection. Close collaboration between treating physicians and microbiologists followed and led to additional investigations, which revealed the diagnosis of a Yersinia pseudotuberculosis infection with extensive lesions in the gastrointestinal tract. Treatment with ciprofloxacin resulted in complete resolution of symptoms and healing of the gastrointestinal lesions. In conclusion, this case underlines the need for a multidisciplinary approach to complex patients of which symptoms have yet to be understood.

[The somewhat different cause of an obturation in stenosing terminal ileitis : Case report of a 39-year-old male patient with Crohn's disease]. / Die etwas andere Ursache einer Obturation bei stenosierender Ileitis terminalis : Fallbericht eines 39-jährigen Patienten mit M. Crohn.

A possible classical complication of Crohn's disease is the formation of a stenosis, which can occur throughout the course of the disease and can present differently depending on the narrowing of the lumen. This article reports the case of a 39-year-old male patient with a stenosing terminal ileitis, which was ultimately only manifested after obstruction by a foreign body.

[Terminal ileitis due to Yersinia enterocolitica: differential diagnosis with Crohn´s disease in a 12-year-old male]. / Ileítis terminal por Yersinia enterocolitica: diagnóstico diferencial con la enfermedad de Crohn en un varón de 12 años.

Yersinia enterocolitica is a gram-negative rod causing intestinal infection in humans. It shows different clinical pictures with many different etiologies to be ruled-out, which sometimes makes it difficult to reach a timely and correct diagnosis. We report the case of an adolescent boy presenting with right lower quadrant pain from terminal ileitis with endoscopic findings akin to Crohn´s disease finally diagnosed as Yersinia enterocolitica, highlighting the usefulness of the different ancillary methods employed.

Yersinia enterocolitica es un bacilo Gram-negativo causante de infección intestinal en los humanos. Se presenta con diferentes cuadros clínicos que obligan a descartar una variedad de etiologías, lo cual, a veces, hace difícil alcanzar un diagnóstico correcto en forma oportuna. Se expone el caso de un varón adolescente con dolor en la fosa ilíaca derecha a partir de una ileítis terminal con hallazgos similares a la enfermedad de Crohn, que se diagnosticó, finalmente, como infección por Yersinia enterocolitica. Se destaca la utilidad de los diferentes métodos auxiliares empleados.

Can microscopic ileitis in patients with clinically suspected inflammatory bowel disease predict the future?

BACKGROUND: The implication of microscopic ileitis finding in patients referred for ileocolonoscopy for clinically suspected inflammatory bowel disease (IBD) is not well defined, and its correlation with clinical outcome has not been fully studied. The current study aims to determine the prognostic yield of biopsies in this setting, and to evaluate the correlation of microscopic ileitis with long-term clinical outcome. METHODS: We reviewed endoscopic reports of patients referred to our department for ileocolonoscopy in the years 2010-2016, as part of a diagnostic work-up for suspected IBD. Patients whose ileocolonoscopies proved normal were included, provided that terminal ileum biopsies had been performed. Accordingly, patients were divided into groups classified as normal (normal or reactive changes) and microscopic ileitis (inflammation or ileitis of any severity). Both groups were followed prospectively to determine clinical outcome. RESULTS: A total of 439 patients met the inclusion criteria. Sixty-four (14.6%) showed inflammation on biopsy and were included in the microscopic ileitis group. Age range and gender figures did not differ significantly between the groups. Overall follow-up period was 6.1 ± 2.3 years. Patients in the microscopic ileitis group were significantly associated with Crohn's diagnosis during the follow-up period compared with the normal group (19% vs 2%, OR = 11.98, 95%CI = 4.48-32.01; p < 0.01). Patients with granuloma or moderate-severe ileitis on biopsy were significantly associated with Crohn's development (100% vs 11%; P < 0.01) compared with mild or nonspecific inflammation. CONCLUSION: The discovery of microscopic ileitis in clinically suspected IBD is associated with increased risk of future diagnosis of Crohn's disease.

Correlation between ultrasound consolidated score and simple endoscopic score for determining the activity of Crohn's disease.

OBJECTIVES: The aim of this study was to develop an ultrasound consolidated score (UCS) in determining the activity of Crohn's disease (CD) and evaluate it with reference to simple endoscopic score (SES). METHODS: From June 2014 to June 2017, 66 patients with CD were retrospectively enrolled in this study. Each patient underwent endoscopy and transabdominal ultrasound (US) examination. The morphological symmetry, echogenicity of bowel wall, bowel wall layer structure, echogenicity of peri-bowel fat, bowel wall thickness (BWT), and Limberg type on power Doppler US were assessed with transabdominal US, and an UCS scoring system was developed based on these characteristics. Endoscopic results were used as the reference standard and SES was calculated to determine the CD activity. Receiver operating characteristic curve analysis was performed to assess the diagnostic performance for determining CD activity and the correlation between UCS and SES was assessed using Spearman correlation analysis. RESULTS: 330 intestinal segments in 66 patients were included. The UCS of the segments in the remission phase ranged from 3.0 to 9.0 (mean, 3.6 ± 0.9) whereas in the active phase from 3.0 to 20.0 (mean, 10.6 ± 4.0) (p < 0.001). The cut-off value of UCS was 6. The associated area under ROC curve, sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 0.980, 88.3%, 95.5%, 93.8%, 91.3%, and 92.3%, respectively. The correlation coefficient between UCS and SES was 0.90, which was higher than the correlation coefficient of 0.83 between BWT and SES. CONCLUSIONS: The newly developed UCS with transabdominal US has a good performance and potentially provides an effective alternative for evaluating the activity of CD. ADVANCES IN KNOWLEDGE: UCS is an effective method to evaluate the activity of CD because it provides comprehensive information of the disease. Therefore, it could be employed as an alternative for diagnosis of CD.

[Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome]. / Ileítis como presentación de linfoma en síndrome de Wiskott-Aldrich.

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.