Cavitating Mesenteric Lymph Node Syndrome With Autoimmune Pancreatitis and Hepatitis: A Case Report and Literature Review

空洞性肠系膜淋巴结综合征合并自身免疫性胰腺炎和肝炎:病例报告及文献综述

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Abstract

Cavitating mesenteric lymph node syndrome (CMLNS) is a rare and severe complication of refractory celiac disease. It is characterized by the triad of cavitating mesenteric lymph nodes, splenic atrophy and villous atrophy of the small bowel mucosa. We present the case of a 36-year-old male patient with a known diagnosis of celiac disease who presented with fever and diarrhea. Further evaluation revealed progression to refractory celiac disease and he developed the rare complication of CMLNS along with concomitant autoimmune hepatitis and autoimmune pancreatitis. We concluded from our case that despite adherence to a strict gluten-free diet and high-dose corticosteroids, cavitary mesenteric lymph node syndrome is highly fatal and leads to complications like septic shock and gastrointestinal perforation. We emphasize the critical need for early recognition of refractory celiac disease and highlight the potential of associated autoimmune disorders, which significantly complicate patient management. Timely diagnosis of complications such as autoimmune pancreatitis and hepatitis is crucial, as they necessitate distinct therapeutic approaches. Our case underlines the urgency of developing more effective treatment strategies to improve patient outcomes.

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