Thymoma-associated autoimmune enteropathy with colonic stricture: a diagnostic and histological challenge

Thymoma-associated autoimmune enteropathy is a rare paraneoplastic syndrome that is characterized by severe chronic diarrhea, malabsorption, and specific histological findings. Because of its rarity, it is difficult to diagnose. We describe an adult patient with protracted diarrhea and weight loss t...

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Bibliographic Details
Published in:Clinical journal of gastroenterology 2022-02, Vol.15 (1), p.112-116
Main Authors: Pieplenbosch, Bo, de Leijer, Jacobus H., van Dop, Willemijn A., Nagtegaal, Iris D., Witteman, Elisabeth M.
Format: Article
Language:English
Subjects:
Abdominal Surgery
Adult
Case Report
Colorectal Surgery
Constriction, Pathologic - etiology
Gastroenterology
Hepatology
Humans
Medicine
Medicine & Public Health
Polyendocrinopathies, Autoimmune - complications
Polyendocrinopathies, Autoimmune - diagnosis
Surgical Oncology
Thymoma - complications
Thymoma - diagnosis
Thymoma - surgery
Thymus Neoplasms - complications
Thymus Neoplasms - diagnosis
Thymus Neoplasms - surgery
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Summary:Thymoma-associated autoimmune enteropathy is a rare paraneoplastic syndrome that is characterized by severe chronic diarrhea, malabsorption, and specific histological findings. Because of its rarity, it is difficult to diagnose. We describe an adult patient with protracted diarrhea and weight loss that developed recurrent sepsis and progressive colonic stenosis due to thymoma-associated autoimmune enteropathy. Ultimately, the diagnosis thymoma-associated autoimmune enteropathy was established by characteristic intestinal histology, anti-enterocytes antibodies, and thorax imaging. Radical thymectomy was performed without improvement. Therapy with azathioprine and infliximab induced clinical remission with complete recovery of the colonic stenosis.
ISSN:1865-7257
1865-7265
DOI:10.1007/s12328-021-01531-2