Nongranulomatous Ulcerative Jejunoileitis with Hypogammaglobulinemia

Corlin, Richard F.; Pops, Martin A.

doi:10.1016/s0016-5085(72)80156-2

Cited by 16 publications

(4 citation statements)

References 10 publications

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“…Six of the survivors come from the coeliac disease group: four are well on a gluten free diet alone (Hindle and Creamer, 1965;Jones and Gleeson, 1973; our case 2); one needs maintenance corticotherapy (Klaeveman et al, 1975) and the last had a second perforation after giving up a gluten free diet (Davidson, 1969). None of the four survivors of the group with possible coeliac disease has fully recovered (Goulston et al, case 4, 1965;Corlin and Pops, 1972;Lotsberg et al, 1976;Cerf et al, 1977). The third group seems to carry the worst prognosis, as only three out of 10 patients were alive at the time of publication (Patterson et al, 1966;Gouffier et al, 1977; our case 4).…”

Section: Relationship With Coeliac Diseasementioning

confidence: 88%

“…Possible CD associated with small bowel ulcerations This group includes 11 patients with total or subtotal villous atrophy of the proximal jejunum but in whom gluten withdrawal had no effect (Shiner, 1963;Goulston et al, case 2 and 3, 1965;Stuber et al, 1971;Corlin and Pops, 1972;Armstrong et al, 1973) or was unappreciable (London et al, 1961;Cerf et al, 1977); a gluten free diet was not prescribed in three of them (Goulston et al, case 1 and 4, 1965;Lotsberg et al, 1976). None of our patients belonged to this group.…”

Section: Relationship With Coeliac Diseasementioning

confidence: 99%

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Chronic non-specific ulcerative duodenojejunoileitis: report of four cases.

Modigliani¹,

Poitras²,

Galian³

et al. 1979

Gut

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SUMMARY Four patients with chronic non-specific ulcerative duodenojejunoileitis (CNSUDJI) are reported. The clinical picture included abdominal pain, fever, and a malabsorption syndrome. Main radiological findings were diffuse narrowing of the jejunal loops with total effacement of the mucosal folds. Multiple peroral biopsies of the small intestine showed various degrees of mucosal abnormalities from total villous atrophy to normal villi, but ulcerations were diagnosed only by operative full thickness biopsies or resection of the small bowel. The ulcerative process was associated with well-documented coeliac disease in two patients: in one of them it occurred as a fatal complication involving also the colon, three years after the start of a gluten free diet, while the disease was in full clinical and histological remission. In the other case, coeliac disease was revealed by obstructive symptoms due to stenosing ulcerations; five months after surgical resection of the stenosis, institution of a gluten free diet induced a dramatic improvement. In the two other patients ulcerations were not associated with coeliac disease: one of them had a patchy villous atrophy and resisted a gluten free diet and total parenteral nutrition; she was improved by and dependent upon steroids but finally died. The last patient had normal villous height; ulcerations were located exclusively along the mesenteric border of the small bowel; he had a low-grade protracted evolution resisting any form of therapy and developed a peripheral neuropathy of unknown aetiology. On the basis of our cases and of a review of the literature the discussion focuses on the difficulty in diagnosing CNSUDJI, its relationship with coeliac disease, and its management and prognosis.

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Section: Relationship With Coeliac Diseasementioning

confidence: 88%

Section: Relationship With Coeliac Diseasementioning

confidence: 99%

Chronic non-specific ulcerative duodenojejunoileitis: report of four cases.

Modigliani¹,

Poitras²,

Galian³

et al. 1979

Gut

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“…Progression to short bowel status was frequent and fatal (7). Some patients had associated severe hypogammaglobulinemia (8,9).…”

Section: Discussionmentioning

confidence: 99%

Extensive Hyperplastic and Ulcerative Mucosal Enteritis in a Child Requiring Total Enterectomy

Besnard,

Peuchmaur,

Mougenot

et al. 1999

J. pediatr. gastroenterol. nutr.

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“…Non-granulomatous ulcerative jejunoileitis is an extremely rare condition. So far only five cases have been reported in the literature 1–5. The disease’s pathology remains largely elusive.…”

mentioning

confidence: 99%

Successful use of TNFα blockade in a severe case of idiopathic non-granulomatous ulcerative jejunoileitis associated with thrombotic thrombocytopenic purpura

Braun¹,

Suárez

Dinter

et al. 2019

BMJ Open Gastroenterol

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We describe the case of 50-year-old female patient who presented with severe gastrointestinal symptoms and progressive weight loss of unknown origin. Shortly after admission, she developed an acute flare of thrombotic thrombocytopaenic purpura (TTP) that had to be treated by plasma exchange therapy and rituximab administration. While the signs of TTP subsided, the gastrointestinal symptoms worsened with abdominal cramps, massive gastric retention, malnourishment and a stenosis due to extensive inflammation and wall thickening of the small bowel. Extensive diagnostic efforts yielded no specific cause, so the patient—based on the histopathological findings—was diagnosed with idiopathic non-granulomatous ulcerative jejunoileitis. Following a highly complicated clinical course over several months, successful remission of the inflammatory activity and recovery of the patient could be obtained by TNF-alpha blockade.

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Nongranulomatous Ulcerative Jejunoileitis with Hypogammaglobulinemia

Cited by 16 publications

References 10 publications

Chronic non-specific ulcerative duodenojejunoileitis: report of four cases.

Chronic non-specific ulcerative duodenojejunoileitis: report of four cases.

Extensive Hyperplastic and Ulcerative Mucosal Enteritis in a Child Requiring Total Enterectomy

Successful use of TNFα blockade in a severe case of idiopathic non-granulomatous ulcerative jejunoileitis associated with thrombotic thrombocytopenic purpura

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