Types of Celiac Disease
1. 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 subtotal villous atrophy.
The typical celiac child displays:
• Persistent diarrhoea
• Large belly (abdominal distension)
• Failure to thrive/weight loss
• Abdominal pain
• Irritability and sleep disturbances
According to current estimates, for each symptomatic case there are approximately six silent or asymptomatic ones.
2. Atypical celiac disease without gastrointestinal symptoms is now considered commoner than the typical kind. It is characterised 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
• Pubertal delay
• Joint pains
• Dermatitis herpetiformis
• Dental enamel hypoplasia of permanent teeth• Hepatitis
• 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 exhibits damaged mucosa (mucosal membrane/lining), positive serology (blood tests) 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 presents minimal change in enteropathy wherein the mucosa has well-formed, normal- looking villi with subtle abnormalities such as an increased number of intra-epithelial 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 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 certain 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 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 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.