{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T18:29:19Z","timestamp":1776968959246,"version":"3.51.4"},"reference-count":19,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2024,9,12]],"date-time":"2024-09-12T00:00:00Z","timestamp":1726099200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/jcm13185417","type":"journal-article","created":{"date-parts":[[2024,9,12]],"date-time":"2024-09-12T10:15:27Z","timestamp":1726136127000},"page":"5417","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Phenotype and Treatment Options for Mesenteric Lymph Node Cavitating Syndrome in Coeliac Disease: A Case Series and Literature Review"],"prefix":"10.3390","volume":"13","author":[{"given":"Federica","family":"Marchetti","sequence":"first","affiliation":[{"name":"Department of Translational Medicine, University of Ferrara, 44121 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suneil A.","family":"Raju","sequence":"additional","affiliation":[{"name":"Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"},{"name":"Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield S10 2RX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4450-2593","authenticated-orcid":false,"given":"Nicoletta","family":"Nandi","sequence":"additional","affiliation":[{"name":"Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"},{"name":"Department of Pathophysiology and Transplantation, University of Milan, 20122 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2713-8355","authenticated-orcid":false,"given":"Mohamed G.","family":"Shiha","sequence":"additional","affiliation":[{"name":"Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"},{"name":"Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield S10 2RX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon S.","family":"Cross","sequence":"additional","affiliation":[{"name":"Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield S10 2RX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4568-0359","authenticated-orcid":false,"given":"Benjamin","family":"Rea","sequence":"additional","affiliation":[{"name":"Department of Clinical Radiology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4244-4529","authenticated-orcid":false,"given":"Giacomo","family":"Caio","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine, University of Ferrara, 44121 Ferrara, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David S.","family":"Sanders","sequence":"additional","affiliation":[{"name":"Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"},{"name":"Division of Clinical Medicine, Faculty of Medicine and Population Health, The University of Sheffield Medical School, Sheffield S10 2RX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1016\/j.revmed.2019.05.010","article-title":"Mesenteric lymph node cavitation in celiac disease: Report of four cases and literature review","volume":"40","author":"Ruch","year":"2019","journal-title":"Rev. Med. Interne"},{"key":"ref_2","first-page":"115","article-title":"Malabsorption and mesenteric cavitation","volume":"17","author":"Hemet","year":"1969","journal-title":"Arch. Anat. Pathol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2991","DOI":"10.3748\/wjg.v16.i24.2991","article-title":"Mesenteric Lymph Node Cavitation Syndrome","volume":"16","author":"Freeman","year":"2010","journal-title":"World J. Gastroenterol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1111\/j.1365-2559.1995.tb01525.x","article-title":"Cavitation of Mesenteric Lymph Nodes: A Rare Complication of Coeliac Disease, Associated with a Poor Outcome","volume":"27","author":"Howat","year":"1995","journal-title":"Histopathology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1007\/s00384-001-0361-8","article-title":"On the Pathogenesis and Clinical Course of Mesenteric Lymph Node Cavitation and Hyposplenism in Coeliac Disease","volume":"17","author":"Schmitz","year":"2002","journal-title":"Int. J. Colorectal Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.2214\/ajr.183.5.1831375","article-title":"Diagnosis of Cavitating Mesenteric Lymph Node Syndrome in Celiac Disease Using MRI","volume":"183","author":"Huppert","year":"2004","journal-title":"AJR Am. J. Roentgenol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1136\/gut.27.6.728","article-title":"Mesenteric Lymph Node Cavitation in Coeliac Disease","volume":"27","author":"Holmes","year":"1986","journal-title":"Gut"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1159\/000517338","article-title":"Hyposplenism and Gastrointestinal Diseases: Significance and Mechanisms","volume":"40","author":"Mathur","year":"2021","journal-title":"Dig. Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/S0140-6736(10)61493-6","article-title":"Post-Splenectomy and Hyposplenic States","volume":"378","author":"Sabatino","year":"2011","journal-title":"Lancet"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1788","DOI":"10.1111\/j.1572-0241.2005.41939.x","article-title":"Depletion of Immunoglobulin M Memory B Cells Is Associated with Splenic Hypofunction in Inflammatory Bowel Disease","volume":"100","author":"Sabatino","year":"2005","journal-title":"Off. J. Am. Coll. Gastroenterol. ACG"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2313","DOI":"10.3748\/wjg.v19.i15.2313","article-title":"Is It Worth Investigating Splenic Function in Patients with Celiac Disease?","volume":"19","author":"Brunetti","year":"2013","journal-title":"World J. Gastroenterol. 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Roentgenol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1111\/j.1440-1746.2011.06991.x","article-title":"Gastrointestinal: Cavitating Mesenteric Lymph Node Syndrome as Manifestation of Anaplastic Large Cell Lymphoma","volume":"27","author":"Wronski","year":"2012","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1136\/flgastro-2019-101191","article-title":"How to Manage Adult Coeliac Disease: Perspective from the NHS England Rare Diseases Collaborative Network for Non-Responsive and Refractory Coeliac Disease","volume":"11","author":"Baggus","year":"2019","journal-title":"Frontline Gastroenterol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"506","DOI":"10.3748\/wjg.v17.i4.506","article-title":"Evaluation of Cladribine Treatment in Refractory Celiac Disease Type II","volume":"17","author":"Tack","year":"2011","journal-title":"World J. Gastroenterol. WJG"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e244692","DOI":"10.1136\/bcr-2021-244692","article-title":"Successful Novel Use of Tofacitinib for Type II Refractory Coeliac Disease","volume":"15","author":"Grewal","year":"2022","journal-title":"BMJ Case Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"840","DOI":"10.1038\/bmt.2010.199","article-title":"Auto-SCT in Refractory Celiac Disease Type II Patients Unresponsive to Cladribine Therapy","volume":"46","author":"Tack","year":"2011","journal-title":"Bone Marrow Transplant."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/18\/5417\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T15:55:02Z","timestamp":1760111702000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/18\/5417"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,12]]},"references-count":19,"journal-issue":{"issue":"18","published-online":{"date-parts":[[2024,9]]}},"alternative-id":["jcm13185417"],"URL":"https:\/\/doi.org\/10.3390\/jcm13185417","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,12]]}}}