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Z Gastroenterol ; 48(9): 1133-7, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20839163

RESUMO

Celiac disease is an immune-mediated enteropathy characterized by mucosal inflammation and villous atrophy of the small bowel upon exposure to ingested gluten. Refractory celiac disease (RCD), defined as persisting villous atrophy with crypt hyperplasia despite strict gluten-free diet, is a rare form of celiac disease with poor prognosis due to a higher rate of severe complications such as life-threatening malnutrition or the development of intestinal T-cell lymphoma. The cavitating mesenteric lymph node syndrome (CMLNS) represents a rare complication of celiac disease with unknown pathogenesis which is associated with but not restricted to RCD and not necessarily associated with a malignant course. We here report a 64-year-old patient who was referred to us with a history of refractory celiac disease. During further diagnostic work-up multiple intraabdominal cystic structures were detected by a computed tomography scan and magnetic resonance imaging. A laparotomy was performed to exclude T-cell lymphoma. Histology of the intraabdominal cysts revealed the diagnosis of cavitating mesenteric lymph node syndrome as the underlying cause of the masses. Steroid therapy was initiated which led to complete regression of diarrhoea but did not induce a diminution of mesenteric lymph nodes. Three years after the diagnosis of CMLNS, the patient presented with an acute abdomen due to a small bowel perforation caused by an enteropathy associated T-cell lymphoma. We discuss the differential diagnoses of intraabdominal masses in celiac disease and review the current literature on CMLNS.


Assuntos
Doença Celíaca/complicações , Doença Celíaca/diagnóstico , Cistos/diagnóstico , Cistos/etiologia , Linfadenite Mesentérica/diagnóstico , Linfadenite Mesentérica/etiologia , Doença Celíaca/tratamento farmacológico , Doença Crônica , Cistos/tratamento farmacológico , Humanos , Masculino , Linfadenite Mesentérica/tratamento farmacológico , Pessoa de Meia-Idade , Doenças Raras/diagnóstico , Doenças Raras/tratamento farmacológico , Doenças Raras/etiologia , Esteroides/uso terapêutico , Falha de Tratamento
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