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Coeliac Disease   by Dr Adrian Morris

(Or Gluten-sensitive Enteropathy)

Coeliac Disease (CD) is a chronic life-long inflammatory disease of the small bowel intestinal lining and may even affect as much as 1% of the UK child population according to the British Medical Journal.  It is triggered by a delayed allergic reaction to a protein called gluten in the normal diet. Gluten is a protein found mainly in Wheat and to a lesser extent in Rye and Barley. Hence the alternative name for the condition of Gluten-sensitive Enteropathy. People with stable Coeliac Disease will tolerate Oats in the diet.

Coeliac Disease (CD) is an inherited disease of the intestinal immune system and affects both men and women. It is highly prevalent in the United Kingdom, affecting 1 in 300 or more people but can be quite difficult to reliably detect. Most CD is finally diagnosed in adulthood usually in the 30-45 year age group. As a result, many cases go undiscovered and are often falsely diagnosed as Irritable Bowel Syndrome (IBS). In fact only about one third of cases are ever diagnosed as CD and appropriately treated with a gluten-free diet. Untreated CD is associated with long-term health risks such as osteoporosis, anaemia and gastrointestinal malignancy.

Samuel Gee first described the condition in 1888. The classical symptoms are malabsorption of food and chronic diarrhoea associated with anaemia, rickets, failure to grow, abdominal bloating, offensive bulky stools, a blistering rash on the buttocks and mouth ulcers. Small children usually become symptomatic when they are weaned off milk onto solids. They present with weight loss, refusal to feed, irritability, abdominal swelling and diarrhoea. However in adults, the disease often presents in an atypical form and may be missed especially with non-specific symptoms such as fatigue, mouth ulcers, skin rashes, vague abdominal pains, intermittent diarrhoea and chronic anaemia.

A rash called Dermatitis Herpetiformis may occasionally occur in Coeliac Disease. It is an intensely itchy rash with symmetrical fine blisters which typically appear on the back, elbows or scalp, almost all people with Dermatitis Herpetiformis have Coeliac Disease.

Certain groups of people are at greater risk for developing Coeliac Disease. It is commoner in individuals from families that already have the condition (being linked to the HLA DQ3 gene). Coeliac Disease is more likely to occur in people with Diabetes on Insulin, Downs Syndrome, Sarcoidosis, Infertility, IgA antibody deficiency and certain autoimmune diseases such as Thyroid disease, Rheumatoid Arthritis, Chronic active Hepatitis, Addisons disease and Sjorgens syndrome of dry eyes and mouth.

How is Coeliac Disease diagnosed?

A recently developed blood test has revolutionised the diagnosis of Coeliac Disease. Up until this point, CD could only be detected after years of severe symptoms and an intestinal biopsy. This resulted in milder cases of Coeliac Disease remaining undiagnosed or being misdiagnosed. The Coeliac Screening blood test measures antibodies in the blood to Gluten or Gliaden in the diet and the damaged Endomysial muscle of the bowel. The Anti-Gliaden (AGA) Antibodies disappear on a Gluten-free diet and are a good indicator of Gluten avoidance and disease resolution. The Endomysial (EMA) Antibodies persist in all people with untreated and treated Coeliac Disease and this was the initial screening test. However, these tests are not 100% accurate in diagnosing CD. 2% of sufferers with IgA deficiency will have a negative EMA test while some people with a positive EMA test may have no CD symptoms at all.  Over the last few years, measuring Tissue Transglutaminase antibodies (to the endomysial antigen) has become the test of choice to compliment the Endomysial antibody test.

We do therefore recommend that people with a positive EMA screening test still have an intestinal biopsy to confirm the presence of CD before embarking on a lifelong diet of wheat and gluten avoidance. The intestinal biopsy can now readily be done at the time of oesophago-gastrointestinal endoscopy (OGD). The small intestinal biopsy must be done after a wheat-based diet for accurate diagnosis. A biopsy specimen taken on a wheat-free diet might show a completely normal intestinal lining. Anti-Reticulin (ARA) Antibodies may also be measured in CD but most UK pathology laboratories tend to test for the Tissue Transglutaminase or Anti-Gliaden IgA and the Anti-Endomysial (EMA) antibodies.

Complications of Coeliac Disease

Coeliac sufferers absorb fewer nutrients from their diet and so may develop nutritional deficiencies. 50% will develop osteoporosis or fractures from calcium deficiency. There is a two-fold increased risk of cancers of the mouth, throat and gullet as well as intestinal lymphomas and carcinomas. They develop chronic anaemia due to not absorbing iron and folic acid from the damaged bowel.  There is a strong link with insulin dependant Diabetes through the HLA DR3 gene. People with Downs Syndrome have a 43 times greater chance of developing Coeliac Disease but will often develop a milder form of the disease.

Treatment of Coeliac Disease

The only effective treatment of CD is strict lifelong complete avoidance of Gluten found in cereals such as Wheat, Rye and Barley (many tolerate Oats). It is completely safe to eat Maize, Corn or Rice. The Coeliac Society of Great Britain is a wonderful support-organisation that provides extensive information on gluten-free diets and foods. Gluten-free flour may be prescribed on the NHS. Unfortunately, CD patients are not entitled to free prescriptions. With the increased potential for developing osteoporosis, sufferers should be closely monitored and all menopausal women and men over 55 years of age who suffer with CD should have bone density (DEXA) scanning.

Final Comment

Coeliac Disease is significantly under-diagnosed in the UK and most cases are detected later in their 40s due to the ill-defined symptoms that occur. The Tissue Transglutaminase screening test is an important development and all patients with vague intestinal symptoms, particularly patients with unexplained diarrhoea, anaemia, mouth ulcers, rashes and chronic fatigue should be tested. It would also be prudent to  screen patients with diabetes, thyroid disease, Downs syndrome and osteoporosis for CD.

For more information visit http://www.coeliac.co.uk

Two conditions may be confused with Coeliac Disease:

Crohns Disease, an autoimmune disease of the small bowel affects people in the 14 to 24 year age groups. Their symptoms are often more severe than in Coeliac Disease. They develop chronic diarrhoea, fever, severe cramps, weight loss, bowel obstruction, vomiting and anorexia. They may have blood in the stools if the lower bowel is affected (20%) of cases. Crohns is also associated with arthritis, anal abscesses, skin lesions and mouth ulcers. Blood tests characteristically show a raised ESR, raised neutrophil blood cell count and raised CRP. The Barium Enema x-ray has a diagnostic appearance. Diet and Gluten play no role in Crohns Disease and treatment is with cortisone and Salazopyrine.

Irritable Bowel Syndrome (IBS) is the "disease of negatives". It affects all ages, predominantly women and is not life-threatening. IBS is diagnosed when there are symptoms of diarrhoea, constipation and abdominal bloating or pain but all tests for other bowel diseases are negative. Those with symptoms who have normal blood tests, normal stool tests, a normal barium enema and a normal colonoscopy end up being diagnosed with IBS.

Two sub-types of IBS occur.

Spastic Colon: Sufferers have mainly left-sided abdominal pain, recurrent constipation and symptom relief on opening the bowels.

Functional diarrhoea: Sufferers have discomfort and bloating associated with frequent diarrhoeal stools in the morning. These are usually watery or mucous laden and can be rather explosive.

 

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