Celiac Disease

Types of Celiac Disease

1. Typical Celiac Disease

Typical Celiac Disease with gastrointestinal symptoms, also called the classical type was at one time, the only picture described in general medical textbooks. Therefore, it was the only one associated with Celiac Disease among clinicians, till recently. It was considered a ‘wasting’ condition, specific to early childhood (six to twenty-four months). Such cases are usually diagnosed easily within the first few years of life. The usual picture of classical Celiac Disease is a fully expressed form of the disease, where the biopsy shows sub-total villous atrophy.

The typical Celiac child displays:
• Persistent diarrhoea
• Large belly (abdominal distension)
• Anorexia
• Failure to thrive/weight loss
• Abdominal pain
• Constipation
• Vomiting
• Irritability and sleep disturbances.

According to current estimates, for each symptomatic case there are approximately six silent or asymptomatic ones.

2. Atypical Celiac Disease

Atypical Celiac Disease without gastrointestinal symptoms is now considered commoner than the typical kind. It is characterized by a combination of a few typical symptoms in association with atypical manifestations. It is commonly seen in adults and the older population.

Atypical Celiac cases may present ‘extra gastrointestinal symptoms’ such as:
• Iron-deficient anaemia, resistant to oral iron
• Soft bones (osteopenia, the precursor to osteoporosis)
• Brittle bones (osteoporosis)
• Short stature
• Lethargy
• Pubertal delay
• Joint pains
• Infertility
• Dermatitis herpetiformis
• Dental enamel hypoplasia of permanent teeth
• Hepatitis
• Arthritis
• Epilepsy with occipital calcifications.

In atypical Celiac Disease, the inflammation and villous atrophy may involve only the upper gastrointestinal tract and may not progress down to the small intestines. Therefore, there may be just one affected nutrient, for example, iron or calcium, which may result in anaemia, osteopenia or osteoporosis. Gastrointestinal symptoms are usually mild but persistent and are often misdiagnosed as irritable bowel syndrome.

3. Silent Celiac Disease

Silent Celiac Disease exhibits damaged mucosa, positive serology and very mild or no gastrointestinal symptoms. It is occasionally discovered following blood (serological) screening in asymptomatic patients such as first-degree relatives or individuals suffering from other autoimmune or genetic disorders such as type-1 diabetes or Down’s syndrome respectively.

Patients with silent Celiac Disease often show symptoms later in life. These are usually in the form of other associated conditions such as dermatitis herpetiformis (red blistering rashes), numbness and tingling in the extremities (peripheral neuropathy), depression, infertility or cancer of the small intestines (adeno-carcinoma). They may feel fatigue or manifest dental enamel defects with or without a marked failure to grow in height.

4. Potential Celiac Disease

Potential Celiac Disease presents minimal change in enteropathy wherein the mucosa has well-formed, normal-looking villi with subtle abnormalities such as an increased number of intraepithelial lymphocytes (IEL). Such patients show a positive result for anti-endomycelial antibodies (AEA) and may have intestinal symptoms. Over time, they may develop a flat mucosa.

5. Latent Celiac Disease

Latent Celiac Disease presents no symptoms and a normal mucosa. Latent Celiac patients may have positive blood tests and negative biopsies, or negative blood tests which either do not warrant a biopsy or show a positive biopsy. Negative tests might turn positive several months or years later. These individuals may show mucosal changes with or without symptoms under the right circumstances and conditions.

This is often seen in first-degree relatives of patients with Celiac Disease or patients with other autoimmune diseases such as diabetes, thyroiditis (hyper or hypothyroidism) and rheumatoid arthritis. Treatment should not be started with a gluten-free diet as it can mask the condition or result in a misdiagnosis, till test results are clearly indicated.

6. Transient Gluten Intolerance

Transient gluten intolerance is a rare condition seen in young children below two years. During the initial diagnosis, these children show gastrointestinal symptoms with severe small intestinal enteropathy. There is a complete remission of symptoms when they are put on a gluten-free diet, and the mucosa returns to normal.

Patients suspected of this condition must undergo regular long-term follow-ups with repeated biopsies, as there is a possibility of a late relapse of Celiac Disease.

7. Dermatitis Herpetiformis

Dermatitis Herpetiformis is a chronic inflammatory skin disease which exhibits itchy, red, blistering skin rashes usually on the outer surfaces of the elbows, buttocks and knees. They may also appear on the shoulder blades, lower spine, face and scalp. The rashes tend to be fairly symmetrically located on the left and right sides of the body. It is usually diagnosed in young or middle-aged adults. In this condition, nearly 90 per cent do not display gastrointestinal symptoms and about 75 per cent show villous atrophy.

It is more common in adults, particularly males, with the male to female ratio being 1.5:1, unlike Celiac Disease, which is more common in females (1:1.5). Dermatitis herpetiformis is not commonly seen in children.

Dermatitis herpetiformis is strongly associated with Celiac Disease and some studies point to both conditions being caused by the same genes. As in Celiac Disease, it is not known why the condition develops at a particular age.

It is treated in the same way as Celiac Disease by maintaining a gluten-free diet for life. It may take six months to achieve moderate improvement in the skin condition and up to two years or more to achieve total control by diet alone, so that medication doses can be progressively reduced and finally eliminated. There are some sulphur-containing drugs, one of which, dapsone, that provide interim, rapid relief of the skin rash and associated itching. These drugs do not have any beneficial effect on the abnormal condition of the small intestines. Therefore, a lifelong gluten-free diet must be followed, to reverse this condition.

8. Wheat sensitivities in Children

1. Parental attitude’s is important. Do not transfer your anxiety to child. Remain calm
2. A positive approach helps with change in family food patterns
3. Young children less than five year old adapt better.
4. Families must be empowered with knowledge.
5. Food must be made palatable and interesting.
6. Support groups help a great deal.
7. Teachers, school authorities and children’s friends must be informed. Children may be subjected to teasing . Take care if situations arise & also prepare your child.
8. School meals and snacks need to be specially modified else arrange for permission to provide packed home food.
9. In joint families all family members and staff must be informed
10. Alternatives to favorite foods must be available
11. Healthy eating must be focused on.Discourage commercially produced processed foods.
12. Avoid introducing wheat, barley, oats and rye till your child is atleast six month. Ideally, wait till the child is one year old. Avoid cow’s milk before one year.
13. Remember play dough ( plasticine) , a common item enjoyed by kids, also contains wheat flour. Watch when children with wheat sensitivity play with it and prevent them from putting it in their mouth. Insist on thoroughly washing their hands after playing
14. Anticipate situation where there can be risk to contamination & take necessary precautions.
15. Celiac wrist band for young children or allergy alert card must be carried.
16. Accidental ingestion of gluten at parties could create panic. Simply avoid repeats.
17. If you are throwing a party, simply serve gluten free food for everyone. This is not a compromise and it can be equally delicious.
18. Watch out for emotional difficulty if your child’s behavior is changing. Don’t hesitate to seek professional help
19. Address other associated condition like type 1 diabetes, thyroid disorders etc.
20. Young teenagers & adolescences can be rebellious. They need special care and understanding. Seek help if needed.
21. Education and communication with your child must be kept up.
22. Encourage them to take control and be in charge as they grow.
23. Teach them to read labels
24. Involve them in making food choices and food preparation.
25. Seek advise on vaccination schedules keeping in mind autoimmunity issues .