Cavitating mesenteric lymph node syndrome: A rare complication of celiac disease. A case report with clinicopathologic features and review of literature

Tinguria, Mukund; Liaconis, Haralambos

doi:10.1016/j.hpr.2021.300580

Cited by 3 publications

(3 citation statements)

References 17 publications

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“…Tinguria and Liaconis reported a case of refractory celiac disease presenting with ulcerative jejunoileitis with intestinal obstruction and cavitating mesenteric lymph node syndrome. 57 The intestinal obstruction required bowel resection. The resected segment of jejunum showed severe luminal narrowing associated with extensive mucosal ulceration.…”

Section: Ulcerative Jejunoileitismentioning

confidence: 99%

“…46,55,56 Tinguria and Liaconis reported a case of refractory celiac disease in a patient who presented with intestinal obstruction secondary to ulcerative jejunitis and cavitating mesenteric lymph node syndrome. 57 Imaging studies are useful in suspecting the diagnosis of CMLNS in patients with celiac disease and confirmation is achieved by pathological evaluation of the excised mesenteric lymph nodes. As similar imaging findings can be seen in a variety of other conditions including mycobacterial infection, Whipple's disease, lymphoma associated with necrosis, as well as necrotic metastatic malignancies in the mesenteric lymph nodes, pathological examination of the lymph nodes is an important part of patient workup.…”

Section: Cavitating Mesenteric Lymph Node Syndromementioning

confidence: 99%

“…As similar imaging findings can be seen in a variety of other conditions including mycobacterial infection, Whipple's disease, lymphoma associated with necrosis, as well as necrotic metastatic malignancies in the mesenteric lymph nodes, pathological examination of the lymph nodes is an important part of patient workup. 57 There are only a few reports describing pathologic features of CMLNS. 20,57,58,59…”

Section: Cavitating Mesenteric Lymph Node Syndromementioning

confidence: 99%

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Rare Complications of Celiac Disease: Clinicopathologic Features

Tinguria¹

2023

MRAJ

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Celiac disease (CD) is an immune mediated disorder characterised by intolerance to glutens in certain grains like whet, barley, and rye. The exposure to gliadin protein component in the susceptible individuals leads to an inflammatory reaction damaging small bowel mucosa with progressive disappearance of intestinal villi. The damaged intestinal mucosa leads to malabsorption. The usual symptoms of celiac disease include diarrhea, steatorrhea, weight loss, fatigue, and abdominal pain. Diagnosis is based on clinical features, duodenal biopsy, elevated levels of anti-gliadin antibodies and response to gluten free diet. Contrary to common belief, celiac disease is a protein systemic disease rather than merely a pure digestive alteration. Celiac disease is closely associated with genes that code HLA -II antigens mainly of DQ2 and DQ8 classes, production of disease specific antibodies (i.e., endomysial antibodies), multiorgan involvement, comorbidity with other autoimmune diseases (shared autoimmunity), familial aggregation, and immune system dysregulation. The clinical presentation of celiac disease can be variable. In mild form, patients can be almost asymptomatic whereas in the most severe form, the patients are at increased risk of life-threatening complications. Celiac disease has a well-known association with other autoimmune diseases such as autoimmune liver diseases (autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis), diabetes mellitus, autoimmune thyroid diseases, skin diseases such as dermatitis herpetiformis, rheumatoid arthritis, systemic lupus erythematosus, Sjogren’s syndrome, psoriasis, sarcoidosis, immune thrombocytopenic purpura, and pancreatitis. In addition, celiac disease may be associated with rare but potentially serious complications such as, collagenous sprue, ulcerative jejunoileitis, refractory celiac disease (RCD), enteropathy associated T-cell lymphoma, small bowel adenocarcinoma (SBA), hyposplenism, and cavitating mesenteric lymph node syndrome (CMLNS). The present article describes clinicopathologic features of these rare but serious complications of celiac disease.

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Section: Ulcerative Jejunoileitismentioning

confidence: 99%

Section: Cavitating Mesenteric Lymph Node Syndromementioning

confidence: 99%

Section: Cavitating Mesenteric Lymph Node Syndromementioning

confidence: 99%

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Rare Complications of Celiac Disease: Clinicopathologic Features

Tinguria¹

2023

MRAJ

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CT and MR Enterography in the Evaluation of Celiac Disease

Penizzotto,

Vespa,

López Grove

et al. 2024

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

Marchetti,

Raju,

Nandi

et al. 2024

JCM

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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.

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Cavitating mesenteric lymph node syndrome: A rare complication of celiac disease. A case report with clinicopathologic features and review of literature

Cited by 3 publications

References 17 publications

Rare Complications of Celiac Disease: Clinicopathologic Features

Rare Complications of Celiac Disease: Clinicopathologic Features

CT and MR Enterography in the Evaluation of Celiac Disease

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

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