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Volume 13
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10.3390/jcm13185417
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Article

Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review

by
Federica Marchetti
1,*,
Suneil A. Raju
2,3,*,
Nicoletta Nandi
2,4,
Mohamed G. Shiha
2,3,
Simon S. Cross
3,
Benjamin Rea
5,
Giacomo Caio
1 and
David S. Sanders
2,3
1
Department of Translational Medicine, University of Ferrara, 44121 Ferrara, Italy
2
Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK
3
Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield S10 2RX, UK
4
Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy
5
Department of Clinical Radiology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK
*
Authors to whom correspondence should be addressed.
J. Clin. Med. 2024, 13(18), 5417; https://doi.org/10.3390/jcm13185417
Submission received: 22 July 2024 / Revised: 23 August 2024 / Accepted: 9 September 2024 / Published: 12 September 2024
(This article belongs to the Section Gastroenterology & Hepatopancreatobiliary Medicine)
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Abstract

Background: There is a paucity of data on mesenteric lymph node cavitation syndrome (MLNCS), a rare condition associated with coeliac disease (CD), characterized by central necrosis within enlarged mesenteric lymph nodes. The largest case series of MLNCS was completed in 1984, (n = 6) and a poor prognosis was identified. Methods: A case series of all patients was conducted with MLNCS treated at the UK NHS England National Centre for Refractory Coeliac Disease between 2000 and 2023. A further literature review was conducted using PubMed and Google Scholar for patients with MLNCS and coeliac disease until 2023. Results: In total, there were 51 patients (6 from our case series and 45 from the literature review); 57% were female, and the mean age was 52.8 years (SD: 14.01 years). The most common presenting symptoms were weight loss (80%) and diarrhea (65%), and patients often had hyposplenism (80%). Persistent villous atrophy was present in 88% of the patients. Ten patients also had Refractory Coeliac Disease. Most of the patients (90%) were on a GFD, but the effect of this is unclear. Treatment with steroids and immunosuppressants resulted in a 40% survival rate. The overall mortality was 43%, associated with cachexia, sepsis, infectious complications, and lymphoma. Conclusions: MLNCS has a poor prognosis, and its diagnosis should prompt further intervention and careful follow-up. Patients commonly present with weight loss and hyposplenism should prompt further investigation. Current treatment options are inadequate and novel therapies are required.
Keywords: coeliac disease; refractory; mesenteric lymph node cavitating syndrome; mortality coeliac disease; refractory; mesenteric lymph node cavitating syndrome; mortality

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MDPI and ACS Style

Marchetti, F.; Raju, S.A.; Nandi, N.; Shiha, M.G.; Cross, S.S.; Rea, B.; Caio, G.; Sanders, D.S. Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review. J. Clin. Med. 2024, 13, 5417. https://doi.org/10.3390/jcm13185417

AMA Style

Marchetti F, Raju SA, Nandi N, Shiha MG, Cross SS, Rea B, Caio G, Sanders DS. Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review. Journal of Clinical Medicine. 2024; 13(18):5417. https://doi.org/10.3390/jcm13185417

Chicago/Turabian Style

Marchetti, Federica, Suneil A. Raju, Nicoletta Nandi, Mohamed G. Shiha, Simon S. Cross, Benjamin Rea, Giacomo Caio, and David S. Sanders. 2024. "Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review" Journal of Clinical Medicine 13, no. 18: 5417. https://doi.org/10.3390/jcm13185417

APA Style

Marchetti, F., Raju, S. A., Nandi, N., Shiha, M. G., Cross, S. S., Rea, B., Caio, G., & Sanders, D. S. (2024). Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review. Journal of Clinical Medicine, 13(18), 5417. https://doi.org/10.3390/jcm13185417

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