the Gastrolab Endoscopy Quiz of the Month - Old Quizes and Answers: October 2001

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The Old Endoscopy Quiz (and Answer) of the Month
October 2001

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Question:


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This middle aged man had lost 20 pounds of his weight during half a year and suffered from diarrhea. These images from the distal bulbus and the descending part of the duodenum are taken after 3 months of PPI therapy. The patient did not use any NSAID´s. Tests for Helicobacter pylori were negative. The first image is from the distal part of the duodenal bulb. The second image shows an area just distal to the papilla and the third image shows the very distal part of the descending duodenum. So, there is an ulceration in the distal duodenal bulb and another large ulceration in the distal descending part of the duodenum. Microscopic examination of the surrounding mucosa showed subtotal villous atrophy. What would be a suitable name for this clinical entity probably not yet described in the medical literature? Any advice concerning treatment?

Answer

Ulcerative coeliac disease.
Please, see comments below

Answers and comments were emailed by

    abubaker elhouni, manchester, uk
    Ervin Toth, Malmö, Sweden
    dongjun fan, taiyuan, china
    Francesco Di Stefano, Sant'Agata li Battiati (CT), Italy
    caio Rossi, santa cruz do sul, rs
    Ahmet TEZEL, Edirne, Turkey


    The center of Rasht, Iran
    Fariborz Mansour ghanaei, Rasht, Iran
    Yekta Kisioglu, Ýstanbul, Turkey
    German Lloret Salom, Alzira, España
    PARABOLI SILVA, Marcio Luiz, CAMPOMOURAO, PARANA-BRAZIL

Pigeon Rocks, Beirut, Lebanon
    Radwan Zahreddine, Beirut, Lebanon

    Comments:

    Coeliac disease is a small bowel mucosal injury caused by gluten in a sensible individual, and responding to glutenfree diet.

    If untreated, the inflammation going in in the mucosa can cause irreversible damage, a "point of no return" is reached, and after that, the response to glutenfree diet is poor or lacking. These patients are severely ill, ulcerations appear in the jejunum and ileum - and in the duodenum in our case - and malignant transformation into a T-cell lymphoma is common. Many somewhat confusing terms have been used considering this condition, e.g. ulcerative jejunitis, ulcerative jejunoileitis, refractory coeliac disease, refractory sprue and enteropathy associated T-cell lymphoma (EATL). Our case shows that the ulcerative lesions can occur also in the duodenum, and our patient was subsequentally found to have a T-cell lymphoma outside the bowel.

    We - as many of You who have answered to this months quiz - would like to suggest the term ulcerative coeliac disease when considering this condition.

    The main features of this condition are:

    - coeliac disease, which has not been treated because the diagnose has never been made before the ulcerative manifestations cause symptoms
    - ulcerative lesions that endoscopically look benign and which can occur anywhere in the small bowel
    - nonresponsiveness to glutenfree diet
    - nonresponsiveness to normal treatment of duodenal ulcer disease
    - nonresponsiveness to other conventional treatments including corticosteroids, making the prognosis poor in many instances
    - a clear association with T cell lymphoma, making this condition a premalignant and in many instances a malignant condition

    Some references concerning refractory coeliac disesase:
     Biagi F, Corazza GR. Defining gluten refractory entropathy. Eur J Gastroenterol Hepatol 2001 May;13(5):561-565
    Mulder CJ, Wahab PJ, Moshaver B, Meijer JW. Refractory coeliac disease: a window between coeliac disease and enteropathy associated T cell lymphoma. Scand J Gastroenterol Suppl 2000;(232):32-37
    Ryan MB. Refractory Celiac Disease. Gastroenterology 2000; 119:243-251