Elemental diet in the treatment of refractory coeliac disease Aditya Mandal and John Mayberry A minority of patients with coeliac disease do not improve in spite of strictly adhering to gluten-free diet and are considered to have developed refractory disease. Such patients are usually treated with steroid and/or immunosuppressive agents but these are not without serious side effects. Herein, we present a patient with refractory coeliac disease in whom remarkable clinical and histological improvement was achieved on elemental diet. Elemental diet should be considered as a treatment option in such patients. Eur J Gastroenterol Hepatol 13:79±80 & 2001 Lippincott Williams & Wilkins European Journal of Gastroenterology & Hepatology 2001, 13:79±80 Keywords: coeliac disease, refractory disease, elemental diet Gastrointestinal Research Unit, Leicester General Hospital, Leicester, UK Correspondence to Dr A. Mandal, Gastrointestinal Research Unit, Leicester General Hospital, Gwendolin Road, Leicester LE5 4PW, UK Tel: 44(0)116 249 0490; fax: 44 (0)116 258 4666; e-mail: adityamandal@hotmail.com Received 19 April 2000 Accepted 16 June 2000 Introduction Coeliac disease is common in the Western European population and has a wide range of clinical presenta- tions. Most patients improve on a gluten-free diet (GFD) but some do not. Although most of these patients will respond to steroids and/or immunosup- pressives, this is not invariably so. These patients pose a dif®cult therapeutic challenge to the clinician. Here we describe a patient who failed to respond to GFD but had a dramatic clinical and histological improve- ment on an elemental diet; we believe this is the ®rst instance of such an approach to be published. Case report A 46-year-old woman presented in July 1996 with marked fatigue and tiredness for the previous few months. She had had these symptoms to a mild extent since the birth of her last child some years previously. Investigations by her general practitioner had revealed iron-de®ciency anaemia which failed to respond to oral iron. She denied any upper or lower gastrointestinal symptoms, in particular there was no history of diar- rhoea. She reported that she had lost a little weight but had always been considered thin. There was no history of non-steroidal anti-in¯ammatory drug use. She was not a vegetarian. Her menstrual periods were regular and she gave no history of menorrhagia. She had loss of libido but no cause had been found on gynaecological review. There was no signi®cant family history, in particular no relative had coeliac disease. On examination, she was thin and pale. She had ®nger clubbing, but no lymphadenopathy or jaundice. Cardio- vascular, respiratory, neurological and abdominal exam- inations were normal. Initial investigations showed a haemoglobin of 10.4 g/dl (normal range 11.5±16.5), white blood cell count 7.4 3 10 9 per litre (normal range 3.6±11.5), platelet count 396 3 10 9 per litre (normal range 140±440), mean corpuscular volume 75.7 ¯ (normal range 80±97). A blood ®lm showed microcytosis and hypochromia. Her renal, thyroid and liver function tests were normal except for a slightly elevated alkaline phosphatase of 282 IU/l (normal range 98±279). Serum iron and folate levels were low, but her serum vitamin B12 level was normal. IgA endomysial and IgG anti-gliadin antibodies were positive. An upper gastrointestinal endoscopy was normal. However, her duodenal biopsies showed com- plete villous atrophy, hyperplasia of the crypts, moder- ate increase of lymphocytes in the lamina propria and a striking increase of intra-epithelial lymphocytes. The histological changes were typical of coeliac disease. A butter fat test was consistent with fat malabsorption and a subsequent small-bowel enema was normal. A diagnosis of coeliac disease (CD) was made and she was started on a GFD. The nature of the disease and its treatment were explained to the patient and she was regularly seen by an experienced dietitian. She also became a member of the Coeliac Society. She felt well on a GFD and her haemoglobin rose to normal levels while her serum alkaline phosphatase fell to the normal range. However, about two years later she again felt lethargic and tired and reported that she had failed to gain weight. Investigations showed a normal full blood count, electrolytes, thyroid, renal and liver function tests. An endomysial antibody was negative. A small- bowel enema did not reveal any abnormality. Although the patient adhered to the GFD rigidly, a repeat duodenal biopsy was consistent with a sub-optimal response. Her lactose tolerance test was normal and a trial of a lactose-free diet (along with the GFD) for a few weeks was without any bene®t. She was reluctant Case report 79 0954±691X & 2001 Lippincott Williams & Wilkins