Clinical Case Summary

We present a case of a 54-year-old male with visceral leishmaniasis mimicking ulcerative colitis clinically and microscopically. Initially the patient presented with unspecific gastroenteritis which resolved in 2 days, with the persisting diarrhea. In the next weeks he reported increased B-symptoms leading to hospital admission. No underlying conditions were reported. Travel history included a visit to Kenia 7 years ago. Further diagnostics showed pancytopenia, slightly elevated inflammation markers, and elevated LDH without hemolysis. An extensive hematological and infectious diseases work-up showed no results. A PET-CT revealed strong FDG uptake in the colon. The subsequent colonoscopy demonstrated high-grade ulcerative inflammation of the large bowel. Histopathology confirmed an ulcerative pancolitis with mucosal remodeling befitting ulcerative colitis. The patient was started on budesonide and mesalazine and was escalated to vedolizumab due to insufficient symptom control. Under this therapy gastrointestinal symptoms ceased, and the patient recovered initially. In follow-up colonoscopy parasitic structures were reported. The PCR for Leishmania from the colon biopsies were positive, with IIFT and ELISA revealing antibodies against Leishmania spp.. The patient was then treated with liposomal amphotericin B for 14 days. Further biopsies showed successful eradication with antibody titers falling. The patients UC therapy was successfully deescalated to budeonside and mesalazine and later all medication was discontinued. Vedolizumab gave the parasites optimal reproduction opportunity which resulted in uncontrolled replication. This however made the diagnosis of the underlying systemic parasitic infection possible as the immune system was able to suppress the parasites without elimination. This highlights the difficulties of diagnosing latent visceral leishmaniasis when presenting with atypical symptoms like mimicking autoimmune bowel diseases like ulcerative colitis.

Disclosure

No conflict of interests

Clinical Case Summary

A 72-year-old man with a 49-year history of ulcerative colitis (UC), characterized as pancolitis and steroid-dependent, underwent routine surveillance colonoscopy. A congested, ulcerated mass was found in the recto-anal region, with no signs of active colitis in the rest of the colon.
From 2000–2012, he was treated with infliximab, discontinued due to secondary failure, followed by azathioprine (2012–2017), which was stopped due to leukopenia. Since then, he has been on vedolizumab. His medical history also includes hypertension and previous tobacco use.
The patient had been in clinical and biochemical remission for the last two years. Given his long-standing UC and the resection of a lateral spreading tumor (LST) via mucosectomy in 2019 showing hyperplastic mucosa with a focus of low-grade dysplasia, he has been under annual endoscopic surveillance.
At the current exam, a large exophytic, ulcerated mass with superficial necrosis and fibrin (Kudo IV) was seen in the recto-anal region, occupying approximately 1/6 of the luminal circumference and bleeding spontaneously.
Histology revealed a poorly differentiated neuroendocrine carcinoma (NEC) with a high proliferative index (Ki-67: 60%). Staging workup, including CT, pelvic MRI, and endoscopic ultrasound, identified a suspicious regional lymph node, that was confirmed by FDG-PET to be metabolically active.
After multidisciplinary discussion, the decision was made to start systemic chemotherapy without delay. If the patient responds, a total proctocolectomy with permanent ileostomy will be considered.
This case is notable for the rarity of recto-anal NECs, especially in UC patients. Their poor prognosis and unclear association with long-term UC or immunosuppressive treatment complicate management and raise important clinical questions.

References

Images that will be uploaded are: endoscopic findings of the colonic mass, histological specimens of the NEC, radiologic findings (PET)

Introduction

Plummer-Vinson syndrome is a rare entity that associates dysphagia, iron deficiency anemia, and cervical esophageal membrane. Our work aimed to describe the clinical profile and to specify the predictive factors of degeneration in patients with Plummer-Vinson syndrome associated with esophageal cancer and to show interest in endoscopic surveillance.

Aims & Methods

It was a retrospective descriptive and analytical study over 30 years (1993-2023) including all patients treated and followed up for Plummer-Vinson syndrome and who presented esophageal cancer. Data collection and statistical analysis were performed by SPSS version 20.0 software.

Results

Out of the 160 patients treated for Plummer-Vinson syndrome, 15 patients had associated esophageal cancer resulting in a prevalence of 9.37%. The median age was 50.6 years with extremes of 28 and 74 years, with a sex ratio of M/F = 2/13. The interval between the diagnosis of Plummer-Vinson syndrome and the appearance of cancer was 8 years +/- 7.4.
All our patients with associated cancer had dysphagia with weight loss, odynophagia in 92.9% (n=13), clinical anemia syndrome in 59 %(n=8), and upper gastrointestinal bleeding in 10%(n=1). All patients had hypoferritinemia with a median value of ferritin 6.2μg/l.
The location of the Plummer-Vinson ring was at the level of the Killian triangle at 46%(n=7) and the upper third of the esophagus at 54%(n=8).
The location of the cancerous process at the level of Killian's triangle was in 46% of cases (n=7), and the location at the level of the upper third of the esophagus was in 19.7%(n=3). In 33.3% of cases (n=5) in the middle third. It was squamous cell carcinoma in all cases. Surgical treatment was initiated in 14% of cases (n=2), radio-chemotherapy in 72% (n=11) and refusal of treatment in 14% of cases (n=2). Two patients progressed well with the disappearance of dysphagia, 10 patients died between 5 months and 5 years of cancer progression, and the rest of the patients could not be followed up.
In multivariate analysis, the statistically significant predictive factors for the degeneration of Plummer-Vinson syndrome were: Age>50.6 years (OR= 2.37, p=0.003 CI=1.875-4.236), Ferritinemia <6.2 μg/l (OR=3.02, p=0.001 CI=0.977-12. 516) and location of the ring at the level of the Killian triangle (OR=1.140, p=0.001 CI=1.057-1.229). Gender and dysphagia did not appear to be associated with degeneration with (p=0.26) and (p=0.35) respectively.

Conclusion

Plummer-Vinson syndrome is a precancerous lesion requiring endoscopic surveillance. In our series, the prevalence was 9.37%. Malignant transformation of this ring at the level of the triangle of Killian was present in 46% of cases. The predictive factors of degeneration were advanced age, low ferritinemia, and the location of the ring in the mouth of Killian.

Introduction

Ulcerative colitis (UC) is increasing in prevalence and incidence in Korea, and each patient shows a different course and treatment response. Since the 2000s, biologics have been developed and treatment options have diversified. Biologics and small-molecule therapeutics for immunological targets are currently under research and development. In the past, the goal was clinical improvement, including symptoms, but now endoscopic mucosal healing and, furthermore, the continuation of histological remission are gradually suggested. Accordingly, the purpose of this study was to identify clinical factors that could predict histological remission in patients receiving a first-line biologic agent, as well as confirm the patient's response to treatment and prognosis.

Aims & Methods

We retrospectively analyzed the medical records of 440 patients who had been diagnosed with UC and exposed to biologics for the first time in our hospital without using biologics at other hospitals between January 01, 2010 and December 31, 2022. Patients who did not undergo pre- and post-exposure endoscopy and biopsy or whose follow-up was discontinued were excluded. Clinical characteristics, blood tests, endoscopic findings, and pathological examination results of a total of 72 patients were collected. Endoscopic response was defined as a decrease in the Mayo Endoscopic Subscore (MES) compared to the score before the first exposure to the biological agent. Histological remission was defined as the result of no cryptitis, no crypt abscess, or no inflammatory cells observed on histological examination, and histological response was defined as remission in patients previously observed with active inflammation.

Results

The ratio of males to females was 53:19, and the median age at the time of diagnosis was 41 years. The average Mayo score at the time of diagnosis was 7.2, and the group with a histological response was 6.0, showing a statistically significant difference. The average albumin was 3.88 and was 4.06 in the histological response group, with a statistically significant difference. The median time to exposure to the first biologic was 1520 days. A colectomy was performed in three cases. Biologics were started with infliximab in 46.7% of patients, followed by adalimumab and vedolizumab in 22.7% and 21.3%, respectively. There was no statistical correlation between histological response and each biologic agent.
MES before exposure to biological agents was 50% in 2 and 44.4% in 3, but there was no statistical difference between each group. Multivariate logistic regression analysis was performed to identify predictors of endoscopic and histological responses after the first exposure to biological agents. As a result, it was confirmed that the Mayo score, ESR, and albumin had a causal relationship with the endoscopic response, respectively, and the odds ratio was 0.781, 1.051, and 3.319, respectively (p-value 0.044, 0.007, 0.049). In addition, it was confirmed that the Mayo score had a causal relationship with the histological response with an odds ratio of 0.796 (p-value 0.046).

Conclusion

This study confirmed that disease activity before treatment was a predictor of histological remission in UC patients using first-line biologics. It was confirmed that the histological response as well as the endoscopic response were well induced when the disease activity was low. Therefore, early escalation to biologics before the patient's condition worsens may be the basis for improving the overall prognosis.

References

Park, Sang Hyoung et al. “Clinical Characteristics and Long-term Prognosis of Elderly-Onset Ulcerative Colitis in a Population-Based Cohort in the Songpa-Kangdong District of Seoul, Korea.” Gut and liver vol. 15,5 (2021): 742-751. doi:10.5009/gnl20289
Park, Sunhee et al. “Histological Disease Activity as a Predictor of Clinical Relapse Among Patients With Ulcerative Colitis: Systematic Review and Meta-Analysis.” The American journal of gastroenterology vol. 111,12 (2016): 1692-1701. doi:10.1038/ajg.2016.418