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

Cavitating mesenteric lymph node syndrome (CMLNS) is a rare but potentially fatal complication of celiac disease (CD) associated with very poor prognosis and mortality reaching up to 50%. Celiac disease is an immune mediated enteropathy characterized by intolerance to ingested glutens. The exposure to gliadin protein component in these susceptible individuals leads to an inflammatory reaction damaging small bowel mucosa with progressive disappearance of intestinal villi. The patients who fail to respond to strict gluten free diet are said to have refractory celiac disease (RCD). This is associated with serious complications such as ulcerative jejunitis as well as enteropathy associated T cell lymphoma. We report on a patient of refractory celiac disease who presented with intestinal obstruction secondary to ulcerative jejunitis and cavitating mesenteric lymph node syndrome. Imaging studies are useful in suspecting the diagnosis of CMLNS in patients with celiac disease and confirmation is achieved by pathological evaluation of 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 work-up. It is important for pathologists to be aware of pathologic features of this rare and potentially fatal complication of celiac disease. This paper reviews the literature and describes clinical, radiological, and pathological features of this rare complication of celiac disease. Possible theories of the pathogenesis of CMLNS are also briefly discussed.