Introduction

Inflammatory bowel disease unclassified (IBDU) is the least common subtype of IBD accounting for around 10% of incident cases in children.¹ It represents a distinct phenotype of colonic IBD where there are insufficient endoscopic and histological patterns to diagnose either ulcerative colitis (UC) or Crohn’s disease (CD).² There is a lack of long-term longitudinal data to fully elucidate the disease course in IBDU. We present nationwide data of an IBDU cohort with long-term (9-20 years) follow-up.

Aims & Methods

In a nationwide study, we analysed a prospectively identified cohort of IBDU patients diagnosed 01/01/03-31/12/13 via all Scottish paediatric IBD centres (Aberdeen, Edinburgh, Glasgow) with follow-up until 01/1/23. Data were obtained retrospectively from electronic medical records (demographics, diagnostic assessment, longitudinal disease severity based on defined global clinician assessment, medical treatment and surgical outcomes) at fixed time points (5-10 years post-diagnosis) and at the last follow-up.

Results

99 patients were identified with IBDU, with 10 excluded as they emigrated out of area. 89 patients were included in the analysis (52/89 (58%) male, median (IQR) age at diagnosis 11.6 (9.2-13.5 years)). All had a diagnosis of IBDU based on the Porto criteria³ using a combination of clinical, endoscopic, histological and radiological findings. The median (IQR) length of follow-up was 10.5 (8.7-14.0 years).
A change of diagnosis was made to UC in 24/89 (27%) and CD in 28/89 (31%) after a median (IQR) disease duration of 3.7 (1.6-7.7 years; 2.5 repeated endoscopies) and 4.38 (1.8-5.6 years; 2 repeated endoscopies) respectively. IBDU remained the diagnosis in 37/89 (42%) after a median (IQR) follow-up of 9.7 (8.0-13.8 years) and a median of 2 (1-2) repeated endoscopies.
At 5 and 10 years of follow-up, 37/83 (45%) and 36/67 (54%) patients were in clinical remission with a reduction in the proportion of patients with moderate to severe disease activity between 5 and 10 years (5-years 32/83 (39%), 10-years 10/67 (15%), p=0.001). No differences were found in remission rates at 5- and 10- years according to disease classification (IBDU vs CD/UC, 5-years p=0.29, 10-years p=0.60). However, compared to IBD-U, patients with active reclassified UC or CD were more likely to have had a moderate-to-severe disease course at 5 -years (IBDU 21% vs CD/UC 50%; p=0.01). 34/89 (38%) patients achieved longstanding clinical remission on aminosalicylates. Escalation to biologics was required in 30/89 (34%) with 7/89 (8%) requiring multiple classes; no differences in biologic use were found according to IBD sub-type (p=0.82).
Resectional surgery was required in 13/89 (15%; 6 UC, 5 CD, 2 IBDU) after a median (IQR) disease duration of 6.7 (1.24-12.7 years). Patients who changed diagnosis to either UC or CD were more likely to require resectional surgery than those remaining IBDU (UC 6/24 (25%) vs IBDU 3/37 (8%), p=<0.001; CD 6/28 (21%) vs IBDU 3/37 (8%), p=<0.001).

Conclusion

This population-based cohort with up to 20 years of follow-up showed that the majority of patients initially diagnosed with IBD-U are reclassified as either UC or CD. Those reclassified are more likely to have a severe disease course with a greater need for surgery.

References

1. Prenzel, F. et al. Frequency of indeterminate colitis in children and adults with IBD-a metaanalysis. J. Crohn’s Colitis 3, 277—281 (2009).
2. Birimberg-Schwartz, L. et al. Development and Validation of Diagnostic Criteria for IBD Subtypes Including IBD-unclassified in Children: a Multicentre Study From the Pediatric IBD Porto Group of ESPGHAN. J. Crohn’s Colitis 11, 1078–1084 (2017).
3. Levine, A. et al. ESPGHAN revised porto criteria for the diagnosis of inflammatory bowel disease in children and adolescents. J. Pediatr. Gastroenterol. Nutr. 58, 795–806 (2014).

Clinical Case Summary

Introduction: Primary epiploic appendagitis (PEA) is a rare cause of acute abdominal pain. The clinical is non-specific, making the diagnosis difficult; hence the interest of CT scan. The preferred treatment is essentially medical.
Objectives: Focus on the particularities of this entity, which can mimic the picture of an acute surgical abdomen, in order to minimize the risk of an unnecessary surgery.
Materials and methods: We present a retrospective and descriptive study of 4 cases diagnosed with PEA, managed in the hepatogastroenterology department in collaboration with the visceral surgery department 2, during the year 2021.
Results:
- The average age of the patients was 50.75 years, with a male predominance.
- Obesity was a predisposing factor for PEA; three patients were overweight.
- The clinical picture was dominated by abdominal pain, found in all patients.
- Associated signs (nausea, vomiting, transit disorders) were rare or absent, found respectively in 2 patients and one patient.
- The clinical examination revealed abdominal tenderness in 3 patients, defensiveness in only one patient, and a temperature higher than 37.3 °Cin 3 patients.
- Laboratory examinations showed hyperleukocytosis in 2 patients and a moderate increase in C-reactive protein in 3 patients.
- CT scan was the key examination for the diagnosis of PEA, performed in all patients.
- Three patients benefited from conservative management, only one patient was operated on.
- The evolution was favorable; no case of recurrence or complication was noted.
Conclusion: PEA is a condition that should be kept in mind as it is managed medically and to spare patients unnecessary surgery or hospitalization.

Introduction

Atrophic gastritis(AG) is mainly caused by Helicobacter pylori(Hp)infection or autoimmunity. Hp-related AG affects both corpus and antral mucosa resulting in multifocal AG(MF-AG), whereas AG driven by autoimmunity is corpus-restricted(CR-AG).
Corpus mucosa gastric atrophy carries an increased risk of gastric dysplasia(GD) and cancer(GC);the neoplastic risk in CR-AG is considered lower, but still debated. GD-GC lesions in AG may arise in the antrum, incisura angularis or, less frequently,the corpus.Data on characteristics and potential differences of GD-GC lesions between MF-AG and CR-AG are lacking.

Aims & Methods

This study aimed to assess clinical,endoscopic,and histological characteristics of GD-GC in MF-AG and CR-AG pts.
A multicenter,cross-sectional study was conducted across 14 Italian GI centers. Data on GD-GC in patients with MF-AG or CR-AG(2001-2023) were retrospectively collected based on clinical,endoscopic, and histological charts: site,dimension,endoscopic,and histologic features of GD-GC, perilesional gastric antral and corpus mucosa(updated Sydney system),and patients’ clinical data (age,sex,outcome after GD-GC diagnosis,Hp infection, parietal cell antibodies(PCA), family history for GC). Inclusion criteria were:patients with MF-AG or CR-AG and GD or GC diagnosis,aged>18 years, availability of endoscopic and histological description of GD-GC and perilesional gastric mucosa, completeness of clinical data.Data were stratified for MF-AG/CR-AG and the GD-GC, perilesional mucosa, and clinical characteristics were compared.

Results

A total of 84 pts[(F46[54.8%];age 70(33-90yrs)] were included. MF-AG and CR-AG were observed in 45(53.6%) and 39(46.4%) pts,respectively. High-grade(HG)-GD,low-grade(LG)-GD,and non-cardia GC were diagnosed in 6(7.1%),31(36.9%), and 47(56.0%)pts.
Age, GC family history,smoking habits, and PCA positivity were similar between MF-AG and CR-AG.Women(69.2% vs 42.2%,p=0.01) and pernicious anemia(29% vs 9.1%,p=0.04) were more frequent in CR-AG than in MF-AG.
The proportion of GD and GC in MF-AG and CR-AG pts was similar:HG-GD was present in 4(8.9%) vs 2(5.1%),LG-GD in 17(37.8%) vs 14(35.9%),GC in 24(53.5%) vs 23(59.0%)pts(p>0.05).
Compared to MF-AG,in CR-AG pts GD-GC were more frequently polypoid lesions (51.6% vs 27.3%,p=0.048) and more frequent in the corpus (55.3% vs 28.6%,p=0.02), but also occurred in the antrum(34.2%) and incisura(10.5%).The intestinal-type GC was the most prevalent and was more frequent in MF-AG than in CR-AG (87%vs60.9%,p>0.046), while diffuse GC was 2 times more frequent in CR-AG than in MF-AG, albeit not reaching statistical significance (26.1% vs 13%, p=0.27).
Concerning GC differentiation grade and staging, no significant differences were observed between MF-AG and CR-AG.Endoscopic treatment of GD-GC was similar in both groups. CR-AG patients underwent surgery more frequently than MF-AG(48.6% vs 23.1%,p=0.0207), but outcome was similar with 90.7% MF-AG and 92.1% CR-AG pts being alive.
Histopathological features of corpus mucosa (acute-chronic inflammation, severity of atrophy,pseudopyloric and intestinal metaplasia) were similar between MF-AG and CR-AG(p>0.05). The histological evidence of Hp in the antrum and corpus was low in both groups (2.3% vs 2.9%,p=0.87).

Conclusion

Non-cardia GC and GD may occur in both,MF-AG and CR-AG, displaying differences in topography and endoscopic presentation, but similarities in perilesional gastric corpus mucosa, differentiation, staging and outcome.
Endo-histological surveillance should be considered in pts with gastric corpus atrophy, irrespective of its etiology and extension.

References

Lahner E, et al. Dig Liver Dis. 2019 Dec;51(12):1621-1632.
Lenti MV, et al. Nat Rev Dis Primers. 2020 Jul 9;6(1):56.
Rugge M, et al. Gut. 2023 Jan;72(1):30-38.

Clinical Case Summary

A 72 years old caucasian women with no relevant medical history presented to the emergency department with two weeks history of abdominal pain in the upper quadrants associated with fullness, vomiting and a significant weight loss and nighttime hypersweating. On physical examination, abdomen was painful on epigastric and periumbilical region. Analytically, hemoglobin 11.1g/dL, leukocytes 9200/mm3, platelet count 315000/μL, CRP 8.22mg/dL. Tests for HIV and EBV were negative. Computed Tomography (CT) of the abdomen and pelvis revealed stomach with thickened walls, densification of adjacent fat and heterogeneous adenopathies. She was admitted to the department of Gastroenterology for investigation. The upper gastrointestinal endoscopy revealed extensive neoplasia of the body and gastric antrum. Biopsies revealed gastric mucosa with infiltration by atypical lymphoid proliferation with a "starry sky" pattern suggestive of high-grade B-cell non-Hodgkin's lymphoma (NHL). FISH analysis revealed t(8;14) c-myc positive. Once morphological and immunohistochemistry feature favors Burkitt’s lymphoma, she was transferred to the department of Hematology. Patient was treated with a full dose CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone) and 3 cycles of dose adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab). Complete remission was documented on positron emission tomography (PET).
Sporadic Burkitt's lymphoma is rare (1-2% of all lymphomas in adults), being one of the most aggressive forms of B-cell NHL. Primary gastric involvement is extremely rare. With the advancement of medicine and greater survival, we must consider this diagnosis, even in the absence of risk factors, atypical location and age group. This case emphasizes the importance of clinicopathological correlation and the importance of early diagnosis for immediate and aggressive intervention, fundamental in reducing morbidity and mortality.

References

1. Zhimin Bai, Yong Zhou. A systematic review of primary gastric diffuse large B-cell lymphoma: Clinical diagnosis, staging, treatment and prognostic factors, Leukemia Research,
Volume 111, 2021, 106716, ISSN 0145-2126, https://doi.org/10.1016/j.leukres.2021.106716.
2. Čubranić A, Golčić M, Fučkar-Čupić D, Brozović B, Gajski D, Brumini I. BURKITT LYMPHOMA IN GASTROINTESTINAL TRACT: A REPORT OF TWO CASES. Acta Clin Croat. 2019 Jun;58(2):386-390. doi: 10.20471/acc.2019.58.02.25. PMID: 31819338; PMCID: PMC6884372.
3. Usman A. Pirzada, Kishore Kumar, Hassan Tariq, Masooma Niazi, Jasbir Makker; Case of Gastric Diffuse Large B-Cell Lymphoma. Case Rep Oncol 6 May 2019; 12 (1): 39–46. https://doi.org/10.1159/000496332
4. Khan A, Khan S, Arshad U. Primary Gastric Burkitt's lymphoma. Pak J Med Sci. 2017 Sep-Oct;33(5):1294-1297. doi: 10.12669/pjms.335.13002. PMID: 29142583; PMCID: PMC5673752.

Introduction

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease in which muscle weakness develops insidiously. In advanced stages of the disease dysphagia, weight loss and malnutrition occur in the majority of patients. Thus, the consideration of Percutaneous Endoscopic Gastrostomy (PEG) tube placement is common. However, the optimal timing and setting for the procedure as well desired spirometry results are either are not stated in most guidelines or debated. Previous studies have suggested greater procedural risk for patients whose forced vital capacity (FVC) was less than 50% than predicted

Aims & Methods

The aim of this study was to assess the safety of our tertiary center simplified approach to PEG tube placement in patients with ALS.
The electronical records of patients who underwent percutaneous endoscopic gastrostomy (PEG) placement between 1.1.2020 to 1.1.2023 were retrospectively reviewed. All adult patients diagnosed with ALS who underwent ambulatory PEG tube placement were included. Patients who underwent PEG tube placement during hospitalization due to acute illness were excluded. The common practice of our center regarding PEG placement in patients with ALS is as follows: initial evaluation at the Gastroenterology clinic of patients referred by the neurology clinic, followed by ambulatory PEG placement under sedation by an anesthesiologist. We collected baseline information from the neurology and gastroenterology clinic visits regarding comorbidities and baseline FVC and the functional rating score [ALS-FRS, 0-48 points, lower score indicating a more severe disease). The data concerning the procedure and possible complications were collected from endoscopy reports, emergency department visits and clinic visits after the procedure.

Results

Six hundred and sixty patients had underwent PEG tube placement in our center during study period. Of them, 67 patients had an established diagnosis of ALS (10.2%). One patient was excluded due to PEG placement in the setting of an acute illness during admission, thus 66 patients were included (mean age at PEG placement 63.5±12.7 years and 37.8% were females). Mean ALS-FRS score was 23.6±8.81 and mean FVC was 49.8±19.45% of predicted. More than half (54.7%) of the patients were using non-invasive ventilation support device at home prior to the procedure and 12.1% of patients had underwent endotracheal tube placement prior to PEG tube placement.
The averaged duration of PEG tube placement was 11.96±5.3 minutes. All PEG procedures were completed successfully. Complications were documented during two PEG placements (3%): one patient suffered from aspiration and was hospitalized and later diagnosed with aspiration pneumonia and the second patient suffered from gastric perforation and peritonitis which was diagnosed a few hours after the procedure. During the 30 days following PEG placement, 3 (4.5%) complications were documented: Two patients (3%) suffered from PEG site infections and were treated with antibiotics and one (1.5%) was diagnosed with aspiration pneumonia. No deaths occurred during the procedure or during the 30 days that followed.

Conclusion

The simple straight forward approach to PEG placement in our institution was successful and safe in the vast majority of cases. The complications rate in our cohort is comparable to other studies, even though the patients in our cohort had significantly reduced lung function and functional scores then accepted in the common practice.

References

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2. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis: Andersen P M, Abrahams S, EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force, Eur J Neurol 2012 Mar;19(3):360-75.
3. Korner S, Hendricks M, Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic options, BMC Neurol 13, 84 (2013).
4. Russ K B, Phillips MC, Percutaneous Endoscopic Gastrostomy in Amyotrophic Lateral Sclerosis, Am J Med Sci . 2015 Aug;350(2):95-7.
5. Gregory S, Siderowf A, Gastrostomy insertion in ALS patients with low vital capacity: respiratory support and survival, Neurology . 2002 Feb 12;58(3):485-7.
6. Thomas K, Schrager J, Percutaneous endoscopic gastrostomy tube placement in amyotrophic lateral sclerosis: a case series with a multidisciplinary, team-based approach, Ann Gastroenterol 2020 Sep-Oct;33(5):480-484