Near Fatal Eosinophilic Gastroenteritis Responding to Oral Sodium Chromoglycate

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Near Fatal Eosinophilic Gastroenteritis Responding to Oral Sodium Chromoglycate Gut: first published as 10.1136/gut.29.9.1282 on 1 September 1988. Downloaded from Gut, 1988, 29, 1282-1285 Case report Near fatal eosinophilic gastroenteritis responding to oral sodium chromoglycate R J MOOTS, P PROUSE, AND J M GUMPEL From Northwick Park Hospital, Harrow, Middlesex SUMMARY Eosinophilic gastroenteritis (EG) is an uncommon disorder, characterised by cramping abdominal pain, diarrhoea and vomiting and histologically by eosinophilic infiltration of bowel wall.' We present a patient who developed EG during the course ofa systemic, necrotising vasculitis, who became critically ill after failure of treatment with corticosteroids and cytotoxic drugs and responded only to oral sodium chromoglycate. Case report bleeding. By then her condition required admission to hospital. A 57 years old cachectic woman was admitted as an On admission she was taking prednisolone (ec) 10 http://gut.bmj.com/ emergency, with a five months history of increasing and 15 mg on alternate days, cyclophosphamide 100 epigastric pains, anorexia, bloody diarrhoea, and mg once a day, spironolactone 50 mg twice a day and weight loss. cimetidine 400 mg twice a day. Her weight was only Ten years earlier, she had been diagnosed as 50 kg after a total weight loss of 20 kg. Clinically, her having a sero negative, non-erosive, symmetrical abdomen was generally distended and there was polyarthritis. A year before this admission she was evidence of gross fluid retention: the peripheral investigated for a painful peripheral neuropathy, neuropathy was unchanged. Initial investigations on September 25, 2021 by guest. Protected copyright. livedo reticularis skin rash and leg ulcers. Non- revealed Hb 107 g/dl, with a lymphocytopaenia and specific vasculitis was present on a skin biopsy. ESR 34 mm/h. Urea, electrolytes, creatinine, Thereafter she became generally unwell developing calcium, phosphate, thyroid and liver function tests mild diarrhoea with weight loss. Liver function was were normal. The serum B12 concentration was low abnormal with mildly raised alkaline phosphatase and a Schilling test showed impaired absorption of and aspartate transaminase. Liver biopsy and colonic B12 with and without intrinsic factor. Other tests biopsy showed a granulomatous vasculitis, involving of small intestine function showed reduced xylose both arteries and veins. She had no history of food absorption and increased faecal fat excretion (7.6 g/ intolerance or allergy. Initial treatment was with 24 h over five days). A C14 labelled breath test was prednisolone and azathioprine. There was little negative. response and cyclophosphamide was substituted for With intravenous hydration and nasogastric azathioprine. After five months therapy which suction, the abdominal pains resolved. Several caused a drug induced lymphocytopaenia, the vascu- attempts at refeeding, even with elemental feeds litis had not improved, the patient's weight had (Vivonex) failed because of recurrence of abdominal dropped by a further 11 kg and she had developed pain and diarrhoea. Her condition deteriorated severe diarrhoea with abdominal pains and rectal further and total parenteral feeding was started. At this stage the gastrointestinal tract was further Address for correspondence: Dr R J Moots, Nuffield Dept. Clinical Medicine John Radcliffe Hospital, Headington, Oxford. investigated. Barium enema showed features of a Received for publication 12 April 1988. pancolitis with a granular mucosa and lack of haustra- 1282 Gut: first published as 10.1136/gut.29.9.1282 on 1 September 1988. Downloaded from Nearfatal eosinophilic gastroenteritis responding to oralsodium chromoglycate 1283 w.eW http://gut.bmj.com/ ,h _ a, -* .4b Figure An operative biopsy of the jejunum showing an inflammatory infiltrate mostly in the submucosa; there is also pneumatosis visible (A). In the inset a high power view ofthe area arrowed is seen, showing that the infiltrate is predominantly eosinophils, appreciated here by their on September 25, 2021 by guest. Protected copyright. bilobed nuclei. (Haematoxylin and eosin). tions. Colonoscopies showed a granular mucosa, resecting the strictures, as the patient's condition with areas of contact bleeding and blue pedunculated continued to worsen. The whole of the small bowel lesions that bled profusely on biopsy. Barium meal looked distended, thickened, and abnormal. The and follow through revealed dilated loops of small large bowel had the appearance of a 'burnt out' bowel with three apparent strictures in the proximal colitis: although no strictures could be found, the and mid ileum. The small bowel transit time was bowel wall felt thick and rigid and it was very difficult grossly increased (24 hours, despite metoclopra- to milk the bowel contents in either direction. Full mide). Atrophic and fibrotic changes were seen on thickness biopsies were taken. Postoperatively, she histological examination of biopsies of colonic and was put on a high protein, high calorie total paren- rectal mucosae, suggesting a resolving ulceration and teral feeding regime, during which her condition reactive amyloid (AA) deposits were found. improved. When oral refeeding was started with a low A laparotomy was done with the intention of fat diet, she felt unwell and the diarrhoea and abdomi- Gut: first published as 10.1136/gut.29.9.1282 on 1 September 1988. Downloaded from 1284 Moots, Prouse, and Gumpel nal pains returned. Despite an increase in steroid eosinophilic ascites, and occasionally pleural dosage there was no improvement in symptoms. effusion. On pathological examination, the serosa Histology of the biopsies taken at laparotomy is thickened, with infiltration of subserosa by showed a dense eosinophilic infiltration of mucosa, eosinophils. and to a lesser extent of muscle (Figure). Also found The tissue damage probably results from infiltra- was pneumatosis intestinalis, and an amyloid infiltra- tion by eosinophils. The toxicity of eosinophil tion (again of the 'AA' type). A diagnosis of EG was granule proteins (especially the major basic protein) therefore established. For this reason, oral sodium to mammalian intestine is well known both in vitro chromoglycate was started at a dose of 100 mg qds. and in vivo,2 and it is likely that these proteins are the Her medication was otherwise unchanged. Within 10 mediators of tissue injury. days, her symptoms gradually improved and she was Unless mucosa is involved, the disease may be allowed home. Over the ensuing 10 weeks the patient particularly hard to diagnose without obtaining full returned to good health, putting on 10 kg in weight. thickness bowel biopsies at laparotomy.34 It should At a follow up clinic visit two months later she was be considered in patients with an appropriate history, well and taking a normal diet: moreover her weight peripheral eosinophilia, normal ESR, and raised had returned to the premorbid level. serum IgE5 although not all these features are always Over the next two and a half years, she has present. In most cases there is no apparent cause; continued to take oral sodium chromoglycate in the occasional causative factors are: herring worms' same dose, and remained free from the diarrhoea, Trichuris trichura,' and other parasites. abdominal pain and distention suffered before. It can be seen that the case presented here fits into Moreover her vasculitis had improved with a return the group EG with predominant mucosal involve- to normal of liver function tests, and she suffered ment. The presence of amyloid and pneumatosis from fewer vasculitic ulcers and these healed up more intestinalis in the intestinal biopsies of this case is quickly than in the past. noteworthy, as these have not been described before in association with EG. Moreover, as this patient had Discussion a non-specific vasculitic disease which appeared to preceed the onset of eosinophilic enteritis, it is Eosinophilic gastroenteritis is a clinicopathological interesting to speculate as to whether the EG arose de entity which is characterised histologically by novo, or as a gastrointestinal manifestation of the eosinophilic infiltration of the gut wall. The patho- systemic vasculitis. Another case has been reported http://gut.bmj.com/ logical process may occur in any part of the gastro- in which a patient with eosinophilic gastroenteritis intestinal tract and may be classified into three later went on to develop polyarteritis nodosa.8 clinical groups, based on the histological site of The conventional treatment of EG is by steroids. eosinophilic infiltration of the bowel wall, although Some cases of steroid resistance have been reported, there is often considerable overlap:' (a) Eosinophilic and were invariably fatal. Sodium chromoglycate has gastroenteritis with predominant mucosal disease. been successfully used in the treatment of allergic This produces symptoms of nausea, vomiting, and conditions such as milk allergy in children,9 gastro- abdominal pain, possibly with a history of allergy. intestinal allergy in adults,'` and malabsorption on September 25, 2021 by guest. Protected copyright. Investigation reveals peripheral eosinophilia, secondary to systemic mastocytosis."1 It has been evidence of malabsorption, and protein losing demonstrated to prevent the release of toxic media- enteropathy, usually with a normal erythrocyte sedi- tors, such as serotonin, histamine, and slow releasing mentation rate. Biopsy of affected intestine reveals a substance of anaphylaxis from mast cell membranes. variable infiltration of mucosa by eosinophils. The This
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