Clinical Case Summary

Background: Strongyloides stercoralis is a clinically inapparent chronic infection in an immunocompetent individual. However, infection by the same parasite in immunosuppressed individuals can lead to hyper infection syndrome and disseminated disease which have an alarmingly high case fatality rate. There have been only case reports of this disease. We here present a case series of Strongyloides stercoralis infection that were diagnosed in our institution.
Methods: We present 5 cases with strongyloidiasis, in immunosuppressed patients.
Results: Most common age group - 40 to 50 years with 90% males. Underlying comorbidities were diabetes, rheumatological disorders. Patients with rheumatological disorders were on steroids or immunomodulators. Most common presenting symptoms -abdominal pain(100%), vomiting(80%), fever(66%), diarrhoea(40%) and pedal edema/ascites(20%). Diagnosis of Strongyloidiasis by Upper GI scopy showing duodenal ulcers/ erosions/ fissuring, with biopsies showing larval and egg forms of the parasite in the duodenal mucosa in all patients. In 70% cases single stool examination failed to detect larva by conventional methods but stool routine micro yielded the diagnosis in one patient. All the patients were prescribed ivermectin and other supportive treatment. No mortality was seen in our study. In various published study the mortality rate for S. stercoralis hyperinfection was 87%, however none of the patients in our study succumb to the infection. This may be related to prompt starting of anti-helmenthic medications along with adequate medical and intensive care wherever needed.
Conclusion: Strongyloides Hyper infection syndrome if treated promptly with adequate medications can yield favorable results even in immunocompromised individuals. In contrast to the available literature suggesting high mortality in hyperinfection syndrome this case series did not record any mortality.

Disclosure

The authors have nothing to disclose

Clinical Case Summary

Background: Cronkhite-Canada syndrome (CCS) is a rare non-hereditary disorder characterized by hamartomatous gastrointestinal polyps, watery diarrhea with severe malabsorption, and dermatological manifestations, including alopecia, onychodystrophy and hyperpigmentation. Approximately 500 cases have been described worldwide, mostly middle-aged Asian men. Its pathophysiology remains elusive, even if inflammation seems to play a pivotal role. Treatment involves nutritional support, immunosuppressive therapy and empirical antibiotics.
Clinical case: A 58-year-old Japanese man was admitted due to watery diarrhea (up to 10 bowel movements/d), abdominal pain, and severe weight loss (15 kg over four months). He presented with dry skin and oral mucosa, dysgeusia, progressive alopecia and onychodystrophy. Fever and night sweats were absent. Past medical and family history were unremarkable and the patient did not receive any medication. Laboratory analyses revealed severe malabsorption with hypophosphatemia, hypocalcemia, hypoalbuminemia and vitamin D deficiency, requiring parenteral nutrition. Infectious and autoimmune enteropathies were ruled out. Endoscopy, including small bowel capsule, showed diffuse mucosal inflammation, multiple gastrointestinal polyps, histologically confirmed as hamartomatous, and an irregular small bowel villous pattern, without evidence of neoplasia. CCS was diagnosed and intravenous prednisolone (1 mg/kg) was started without significant improvement. Subsequently, Infliximab (5 mg/kg) was administered for four months. Due to insufficient clinical response, empirical therapy with Upadacitinib 45 mg/d was initiated (later reduced to a maintenance dose of 30 mg/d), resulting in clinical and endoscopic improvement, which still persist after seven months.
Conclusion: Given the lack of standardized treatment for CCS, this case shows the potential role of JAK inhibitors as an effective therapeutic option after corticosteroids and anti-TNF therapy failure.

References

Figures:

• Captures from EGD, colonoscopy and SBCE pre- and post-therapy with JAK-inhibitors.
• Pictures showing alopecia and onychodystrophia.

1. Cronkhite LW, Jr., Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med. 1955;252:1011–5
2. Hu H, Wu Y, Zhang Y, Zhang L, Zhang J, Zhang R. Comprehensive treatment of Cronkhite-Canada syndrome: A case report and literature review. Medicine (Baltimore). 2023 Feb 10;102(6):e32714. doi: 10.1097/MD.0000000000032714.
3. Ze-Yu Wu, Li-Xuan Sang, Bing Chang, Cronkhite–Canada syndrome: from clinical features to treatment, Gastroenterology Report, Volume 8, Issue 5, October 2020, Pages 333–342, https://doi.org/10.1093/gastro/goaa058
4. Sweetser S, Alexander GL, Boardman LA. A case of Cronkhite-Canada syndrome presenting with adenomatous and inflammatory colon polyps. Nat Rev Gastroenterol Hepatol. 2010 Aug;7(8):460-4. doi: 10.1038/nrgastro.2010.112.
5. Mizushima H, Kinugasa H, Otsuka M. Unraveling a Rare Case: Diarrhea, Alopecia, and Polyposis. Gastroenterology. 2025 Feb 18:S0016-5085(25)00371-5. doi:10.1052/j.gastro.2025.02.005.
6. Wallenhorst T, Pagenault M, Bouguen G, Siproudhis L, Bretagne JF. Small-bowel video capsule endoscopic findings of Cronkhite-Canada syndrome. Gastrointest Endosc. 2016 Oct;84(4):739-40. doi: 10.1016/j.gie.2016.04.004.

Introduction

Cases of autochthonous Strongyloides stercoralis infection have been reported rarely in Northern Italy,. There are no reports from Southern Italy. Since S. stercoralis may harbor in dogs and other farm animals, we aimed to investigate the prevalence of such infestation in farm workers in Southern Italy.

Aims & Methods

Capillary blood samples were drawn from farm and doghouse workers in the county area of Bari (South Italy). Screening test was a commercial ELISA test (Strongyloides ratti by Bordier Affinity Products SA). Serology-positive patients were offered Ivermectin therapy, single dose of 200 ug/Kg. Patients underwent, at baseline and 4 months after therapy, clinical examination; severity of abdominal pain, diarrhea, skin rash, urticaria, asthma symptoms were estimates by a 10-point VAS scale. Stool consistency was classified according to the Bristol scale. Full blood count was also performed both at baseline and follow up. Data were expressed as median with interquartile range (IQR). Wilcoxon signed rank test was used for statistical analysis.

Results

Among 120 workers, 6 patients were serology- positive, with a prevalence of 5%. Two of them worked in doghouses. Abdominal pain was the most frequent symptom (3 patients, 50% cases), which resolved after therapy in all patients with VAS from 1 (IQR 0-5.8) to 0 (IQR 0-0), p=0.25. No variation in stool consistency was recorded (p=1). Two patients complained of urticaria, which totally resolved in one subject and improved in the other one. Asthma was referred bytwo patients, with full clearancein one of them. We observed a non statistically significant trend of eosinophil count reduction, from 190/mm³ (IQR 140-240) to 180/mm³ (IQR 150-220), p=0.14.

Conclusion

In our area, the prevalence of the parasitosis is low. Therapy may be helpful to reduce the severity of some symptoms such as abdominal pain, asthma and urticaria. A marginal effect on eosinophils has been observed.

Clinical Case Summary

Seronegative enteropathies pose a complex diagnostic challenge, as their histology mimics celiac disease (CeD) (1). We report a 51-year-old patient with hypertension and osteoporosis, referred for abdominal pain, weight loss, and constipation. Blood tests showed no anemia; anti-transglutaminase and anti-endomysium antibodies were negative, but HLA typing was compatible with CeD. Ileocolonoscopy excluded IBD and microscopic colitis, while OEGD revealed villous atrophy (MARSH 3c). Negative anti-enterocyte antibodies, and exclusion of drugs and infections, led to a diagnosis of seronegative CeD. A gluten-free diet was started, but after 1 year, symptoms improved only partially despite good adherence. OEGD showed persistent duodenal damage with increased intraepithelial lymphocytes (IELs), CD3+ and CD8+ T-cells with monoclonal TCR expression (fig. 1), consistent with RCeD type 1, despite no overt malabsorption. The patient began prednisone 50 mg/day, then switched to open-capsule budesonide, with some clinical benefit but persistent atrophy.
A year later, his daughter was diagnosed with CVID and a heterozygous STAT3 variant (c.1252T>C, p.Cys418Arg), somatically acquired in NK-cell leukemia. A luciferase assay confirmed its gain-of-function (GOF) effect (fig. 2). Family studies revealed the same germline variant in the patient. His diagnosis was revised to STAT3 GOF-associated enteropathy. Further investigations revealed hypogammaglobulinemia, splenomegaly, bronchiectasis, and emphysema. Treatment with Ruxolitinib (a JAK1/2 inhibitor) was started, resulting in clinical and histological remission (fig. 3).
STAT3 GOF enteropathy is a very rare condition (2,3) that should be considered in the differential diagnosis of seronegative villous atrophy. Anti-JAK therapy may be effective (4).

References

References of the images:
Figure 1: Histologic aspects at diagnosis
Figure 2: Luciferase assay of STAT3
Figure 3: Histologic aspects after 1 year of therapy

1. Schiepatti A, Sanders DS, Baiardi P, Caio G, Ciacci C, Kaukinen K, et al. Nomenclature and diagnosis of seronegative coeliac disease and chronic non-coeliac enteropathies in adults: the Paris consensus. Gut. 2022 Jun 8;71(11):2218–25.
2. Vogel TP, Leiding JW, Cooper MA, Forbes Satter LR. STAT3 gain-of-function syndrome. Vol. 10, Frontiers in Pediatrics. Frontiers Media SA; 2023.
3. Flanagan SE, Haapaniemi E, Russell MA, Caswell R, Allen HL, De Franco E, et al. Activating germline mutations in STAT3 cause early-onset multi-organ autoimmune disease. Nat Genet. 2014;46(8):812–4.
4. Kaneko S, Sakura F, Tanita K, Shimbo A, Nambu R, Yoshida M, et al. Janus kinase inhibitors ameliorate clinical symptoms in patients with STAT3 gain-of-function. Immunotherapy Advances. 2023;3(1).

Clinical Case Summary

Case presentation: An 18-year-old male with a four-year history of recurrent nausea, vomiting, and upper abdominal pain, worsened after meals, was diagnosed with Superior Mesenteric Artery Compression Syndrome (SMAS).
Diagnosis and treatment: The diagnosis of SMAS was confirmed by CT and CTA, which revealed a reduced aortomesenteric angle and duodenal compression, excluding other gastrointestinal conditions like peptic ulcers and pancreatitis. An upper GI contrast study showed delayed gastric transit, and endoscopy revealed mild mucosal edema without obstruction. Blood tests showed elevated WBC (14.99 × 10⁹/L) and low albumin (61.5 g/L), indicating inflammation and malnutrition.
Given the failure of conservative treatment, the patient underwent laparoscopic duodenojejunostomy on March 14, 2025. The surgery was successful with minimal blood loss. Postoperatively, the patient received somatostatin, antibiotics, and nutritional support, with gradual transition from parenteral to enteral feeding. Follow-up imaging confirmed a successful anastomosis, and the patient experienced significant symptom relief and improved nutritional status.
Outcome and discussion: The patient was discharged after 38 days with substantial improvement in symptoms and quality of life. This case highlights the importance of considering SMAS in young patients with persistent postprandial symptoms. Early surgical intervention, such as duodenojejunostomy, is effective for relieving duodenal compression and improving outcomes, particularly when conservative treatment fails.The poster will include the patient's abdominal enhanced CT scan, 3D reconstruction images, pathological images, and postoperative drainage volume changes.

Clinical Case Summary

We report a rare case of recalcitrant gut and cutaneous Sweet Syndrome(SS) which was successfully treated with a novel not reported before treatment regime, of Adalimumab and Methotrexate (MTX).
A 50-year-old postmenopausal female with quiescent cutaneous SS presented with symptomatic iron deficiency anaemia. Thorough endoscopic gut evaluation revealed ileal ulcers with dense neutrophil infiltrates with no evidence of inflammatory bowel disease (IBD) on histology. A detailed drug history, infection and malignancy screens were unyielding. She was commenced on Azathioprine (AZT) and Prednisolone for gut SS. Six months later, her hematological indices remained low. Repeat enteroscopy showed persistent mid ileal ulcers. In view of disease recalcitrance, she was switched to MTX with Prednisolone however she represented to hospital for melena. Video capsule endoscopy showed similar findings of ulcers in the mid-distal small bowel. She remained refractory to treatment with continued abdominal pain and weight loss and persistent iron deficiency anaemia with raised inflammatory indices. We decided to commence her on a novel combination of an anti-tumour necrosis factor (TNF) agent, Adalimumab with MTX, based on extrapolation of data from previously published case reports of recalcitrant cutaneous SS and IBD (1-3). MTX was included to reduce immunogenicity (4). She tolerated Adalimumab well with Prednisolone and MTX being gradually tapered. Five months later, repeat enteroscopy showed complete healing of ulcers with resolution of constitutional symptoms and improvement in biochemical indices.
This case presents both a diagnostic and therapeutic challenge in the management of a very rare condition, in which conventional immunosuppressive agents were unsuccessful. A diagnosis of gut SS should be considered in the presence gut ulcers, especially if histological features of IBD are absent. In recalcitrant cases anti-TNF may be an alternative treatment option.

References

1. Agarwal A, Barrow W, Selim MA, Nicholas MW. Refractory Subcutaneous Sweet Syndrome Treated With Adalimumab. JAMA Dermatology. 2016;152(7):842. doi:https://doi.org/10.1001/jamadermatol.2016.0503
2. Calabrese L, Caldarola G, Peris K, De Simone C. Recalcitrant Sweet Syndrome successfully treated with adalimumab. JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 2020;19(1):122-124. doi:https://doi.org/10.1111/ddg.14223
3. Knöpfel N, Theiler M, Luchsinger I, et al. Infliximab for the treatment of recalcitrant bullous Sweet syndrome in a 10‐year‐old girl. Pediatric Dermatology. 2020;37(6):1183-1184. doi:https://doi.org/10.1111/pde.14356
4. Jani M, Barton A, Warren RB, Griffiths CEM, Chinoy H. The role of DMARDs in reducing the immunogenicity of TNF inhibitors in chronic inflammatory diseases. Rheumatology. 2013;53(2):213-222. doi:https://doi.org/10.1093/rheumatology/ket

1. Histology slides of initial biopsy slide of small bowel ulcer showing neutrophil infiltrate -> after AZT -> after MTX -> after Adalimumab
2. Endoscopic ulcer pictures at diagnosis -> after AZT -> after MTX -> after Adalimumab