Celiac Disease

Definition:
Celiac disease, sometimes  called celiac sprue, is a digestive disorder that occurs when an individual’s immune system overreacts to the protein gluten, or other proteins within gluten such as gliadin, found in grains including wheat, rye, barley, and to some degree, oats. When a patient with the disease eats food that contains gluten, the immune system’s response damages the intestinal lining. This causes symptoms of abdominal pain and bloating after consuming gluten.

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You may click to see the picture…> Coeliac disease :Classification and external resources

Diagram to show the different stages of Coeliac Disease

It  is an autoimmune disorder of the small intestine that occurs in genetically predisposed people of all ages from middle infancy onward. Symptoms include chronic diarrhoea, failure to thrive (in children), and fatigue, but these may be absent, and symptoms in other organ systems have been described. A growing portion of diagnoses are being made in asymptomatic persons as a result of increased screening. Coeliac disease is caused by a reaction to gliadin, a prolamin (gluten protein) found in wheat, and similar proteins found in the crops of the tribe Triticeae (which includes other cultivars such as barley and rye). Upon exposure to gliadin, and certain other prolamins, the enzyme tissue transglutaminase modifies the protein, and the immune system cross-reacts with the small-bowel tissue, causing an inflammatory reaction. That leads to a truncating of the villi lining the small intestine (called villous atrophy). This interferes with the absorption of nutrients, because the intestinal villi are responsible for absorption. The only known effective treatment is a lifelong gluten-free diet. While the disease is caused by a reaction to wheat proteins, it is not the same as wheat allergy.

This condition has several other names, including: cœliac disease (with œ ligature), c(o)eliac sprue, non-tropical sprue, endemic sprue, gluten enteropathy or gluten-sensitive enteropathy, and gluten intolerance. The term coeliac derives from the Greek  (koiliak?s, “abdominal”), and was introduced in the 19th century in a translation of what is generally regarded as an ancient Greek description of the disease by Aretaeus of Cappadocia.

No treatment can cure celiac disease. However, you can effectively manage celiac disease through changing your diet.


Symptoms :-

Severe coeliac disease leads to the characteristic symptoms of pale, loose and greasy stool (steatorrhoea), weight loss or failure to gain weight (in young children). People with milder coeliac disease may have symptoms that are much more subtle and occur in other organs rather than the bowel itself. Finally, it is possible to have coeliac disease without any symptoms whatsoever. Many adults with subtle disease only have fatigue or anaemia.

There are no typical signs and symptoms of celiac disease. Most people with the disease have general complaints, such as:
*Intermittent diarrhea
*Abdominal pain
*Bloating

Sometimes people with celiac disease may have no gastrointestinal symptoms at all. Celiac disease symptoms can also mimic those of other conditions, such as irritable bowel syndrome, gastric ulcers, Crohn’s disease, parasite infections, anemia, skin disorders or a nervous condition.

Celiac disease may also present itself in less obvious ways, including:

*Irritability or depression
*Anemia
*Stomach upset
*Joint pain
*Muscle cramps
*Skin rash
*Mouth sores
*Dental and bone disorders (such as osteoporosis)
*Tingling in the legs and feet (neuropathy)

Some indications of malabsorption that may result from celiac disease include:

*Weight loss
*Diarrhea
*Abdominal cramps, gas and bloating
*General weakness and fatigue
*Foul-smelling or grayish stools that may be fatty or oily
*Stunted growth (in children)
*Osteoporosis
*Anemia

Another gluten-related condition :-
Dermatitis herpetiformis is an itchy, blistering skin disease that also stems from gluten intolerance. The rash usually occurs on the elbows, knees and buttocks. Dermatitis herpetiformis can cause significant intestinal damage identical to that of celiac disease. However, it may not produce noticeable digestive symptoms. This disease is treated with a gluten-free diet, in addition to medication to control the rash.

When to see a doctor :-
If you notice or experience any of the signs or symptoms common to celiac disease, see your doctor. If someone in your family is known to have celiac disease, you may need to be tested.

Seek medical attention for a child who is pale, irritable, fails to grow, and who has a potbelly, flat buttocks and malodorous, bulky stools. Other conditions can cause these same signs and symptoms, so it’s important to talk to your doctor before trying a gluten-free diet.

Causes:-
General: When a celiac patient eats gluten, or other protein components of gluten, such as gliadin, the body’s immune system overreacts. Gluten is present in all types of wheat (including farina, graham flour, semolina, and durum), barley, rye, bulgur, Kamut, kasha, matzo meal, spelt, and triticale. The gluten is mistaken for a harmful invader, such as bacteria, and an attack is launched. Immune system cells flood to the stomach and intestine to destroy the gluten. However, among these immune cells are autoantibodies that attack the lining of the intestine by mistake. As a result, the intestinal lining becomes damaged.

 

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Gliadin is a protein component of gluten, found in wheat and several other cereal grains of the genus Triticum. Patients with celiac disease are sensitive to the  and forms of gliadins. In response to gliadin, anti-gliadin IgA antibodies are produced, which are reportedly found in many patients with celiac disease.

Inherited: Researchers believe that many cases of celiac disease are inherited (passed down through families). Researchers estimate that if someone in a patient’s immediate family (parent or sibling) has celiac disease, the patient has a 5-15% chance of developing the disease as well.

Trauma: It also appears that many cases of celiac disease develop after trauma, such as an infection, stress, physical injury, surgery, or pregnancy.

Other disorders: Celiac disease is associated with autoimmune disorders. Autoimmune disorders occur when the immune system attacks the body by mistake. Autoantibodies in the blood bind to components of an individual’s own cells, triggering other cells to attack the body. The most common autoimmune disorders associated with celiac disease are lupus erythematosus, type I diabetes, rheumatoid arthritis, thyroid disease, and microscopic colitis (disorder that causes inflammation of the colon).

Risk factors:-
Although celiac disease can affect anyone, it tends to be more common in people who have:

*Type 1 diabetes
*Autoimmune thyroid disease
*Down syndrome
*Microscopic colitis, particularly collagenous colitis

Additionally, certain genes — HLA-DQ2 and DQ8 — are associated with an increased risk of celiac disease. But, experts also suspect that other, as yet unknown, genes also play a role in the development of celiac disease.

Complications:-
Left untreated, celiac disease can lead to several complications:

*Malnutrition. Untreated celiac disease can lead to malabsorption, which in turn can lead to malnutrition. This occurs in spite of what appears to be an adequate diet. Because vital nutrients are lost in the stool rather than absorbed in the bloodstream, malabsorption can cause a deficiency in vitamins and minerals, such as B-12, D, folate and iron, resulting in anemia and weight loss. Malnutrition can cause stunted growth in children and delay their development.

*Loss of calcium and bone density. With continued loss of fat in the stool, calcium and vitamin D may be lost in excessive amounts. This may result in a bone disorder called osteomalacia, a softening of the bone also known as rickets in children, and loss of bone density (osteoporosis), a condition that leaves your bones fragile and prone to fracture. In addition, lack of calcium absorption can lead to a certain type of kidney stone (oxalate stone).

*Lactose intolerance. Because of damage to your small intestine from gluten, foods that don’t contain gluten also may cause abdominal pain and diarrhea. Some people with celiac disease aren’t able to tolerate milk sugar (lactose) found in dairy products, a condition called lactose intolerance. If this is the case, you need to limit food and beverages containing lactose as well as those containing gluten. Once your intestine has healed, you may be able to tolerate dairy products again. However, some people may continue to experience lactose intolerance despite successful management of celiac disease.

*Cancer. People with celiac disease who don’t maintain a gluten-free diet also have a greater chance of getting one of several forms of cancer, especially intestinal lymphoma and bowel cancer.

*Neurological complications. Celiac disease has also been associated with disorders of the nervous system, including seizures (epilepsy) and nerve damage (peripheral neuropathy).

Diagnosis:
There are several tests that can be used to assist in diagnosis. The level of symptoms may determine the order of the tests, but all tests lose their usefulness if the patient is already taking a gluten-free diet. Intestinal damage begins to heal within weeks of gluten being removed from the diet, and antibody levels decline over months. For those who have already started on a gluten-free diet, it may be necessary to perform a re-challenge with some gluten-containing food in one meal a day over 2–6 weeks before repeating the investigations.

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Combining findings into a prediction rule to guide use of endoscopy reported a sensitivity of 100% (it would identify all the cases) and specificity of 61% (it would be incorrectly positive in 39% of those without the disease, not a false positive rate of 39%). The prediction rule recommends that patients with high-risk symptoms or positive serology should undergo endoscopy. The study defined high-risk symptoms as weight loss, anaemia (haemoglobin less than 120 g/l in females or less than 130 g/l in males), or diarrhoea (more than three loose stools per day).

Blood tests:-
Serological blood tests are the first-line investigation required to make a diagnosis of coeliac disease. Serology for anti-tTG antibodies has superseded older serological tests and has a high sensitivity (99%) and specificity (>90%) for identifying coeliac disease. Modern anti-tTG assays rely on a human recombinant protein as an antigen. An equivocal result on tTG testing should be followed by antibodies to endomysium.

Because of the major implications of a diagnosis of coeliac disease, professional guidelines recommend that a positive blood test is still followed by an endoscopy/gastroscopy and biopsy. A negative serology test may still be followed by a recommendation for endoscopy and duodenal biopsy if clinical suspicion remains high due to the 1 in 100 “false-negative” result. As such, tissue biopsy is still considered the gold standard in the diagnosis of coeliac disease.

Historically three other antibodies were measured: anti-reticulin (ARA), anti-gliadin (AGA) and anti-endomysium (EMA) antibodies. Serology may be unreliable in young children, with anti-gliadin performing somewhat better than other tests in children under five. Serology tests are based on indirect immunofluorescence (reticulin, gliadin and endomysium) or ELISA (gliadin or tissue transglutaminase, tTG).

Guidelines recommend that a total serum IgA level is checked in parallel, as coeliac patients with IgA deficiency may be unable to produce the antibodies on which these tests depend (“false negative”). In those patients, IgG antibodies against transglutaminase (IgG-tTG) may be diagnostic.

Antibody testing and HLA testing have similar accuracies. However, widespread use of HLA typing to rule out coeliac disease is not currently recommended.

Endoscopy:-Click to see the picture
An upper endoscopy with biopsy of the duodenum (beyond the duodenal bulb) or jejunum is performed. It is important for the physician to obtain multiple samples (four to eight) from the duodenum. Not all areas may be equally affected; if biopsies are taken from healthy bowel tissue, the result would be a false negative.

Most patients with coeliac disease have a small bowel that appears normal on endoscopy; however, five concurrent endoscopic findings have been associated with a high specificity for coeliac disease: scalloping of the small bowel folds (pictured), paucity in the folds, a mosaic pattern to the mucosa (described as a “cracked-mud” appearance), prominence of the submucosa blood vessels, and a nodular pattern to the mucosa.

Until the 1970s, biopsies were obtained using metal capsules attached to a suction device. The capsule was swallowed and allowed to pass into the small intestine. After x-ray verification of its position, suction was applied to collect part of the intestinal wall inside the capsule. One often-utilised capsule system is the Watson capsule. This method has now been largely replaced by fibre-optic endoscopy, which carries a higher sensitivity and a lower frequency of errors.


Pathology
:-
The classic pathology changes of coeliac disease in the small bowel are categorised by the “Marsh classification”

*Marsh stage 0: normal mucosa
*Marsh stage 1: increased number of intra-epithelial lymphocytes, usually exceeding 20 per 100 enterocytes
*Marsh stage 2: proliferation of the crypts of Lieberkuhn
*Marsh stage 3: partial or complete villous atrophy
*Marsh stage 4: hypoplasia of the small bowel architecture

Marsh’s classification, introduced in 1992, was subsequently modified in 1999 to six stages, where the previous stage 3 was split in three substages. Further studies demonstrated that this system was not always reliable and that the changes observed in coeliac disease could be described in one of three stages—A, B1 and B2—with A representing lymphocytic infiltration with normal villous appearance and B1 and B2 describing partial and complete villous atrophy.

The changes classically improve or reverse after gluten is removed from the diet. However, most guidelines don’t recommend a repeat biopsy unless there is no improvement in the symptoms on diet. In some cases, a deliberate gluten challenge, followed by biopsy, may be conducted to confirm or refute the diagnosis. A normal biopsy and normal serology after challenge indicates the diagnosis may have been incorrect.

Other diagnostic tests:-
At the time of diagnosis, further investigations may be performed to identify complications, such as iron deficiency (by full blood count and iron studies), folic acid and vitamin B12 deficiency and hypocalcaemia (low calcium levels, often due to decreased vitamin D levels). Thyroid function tests may be requested during blood tests to identify hypothyroidism, which is more common in people with coeliac disease.

Osteopenia and osteoporosis, mildly and severely reduced bone mineral density, are often present in people with coeliac disease, and investigations to measure bone density may be performed at diagnosis, such as dual energy X-ray absorptiometry (DXA) scanning, to identify risk of fracture and need for bone protection medication.

Screening:-
Due to its high sensitivity, serology has been proposed as a screening measure, because the presence of antibodies would detect previously undiagnosed cases of coeliac disease and prevent its complications in those patients. There is significant debate as to the benefits of screening. Some studies suggest that early detection would decrease the risk of osteoporosis and anaemia. In contrast, a cohort study in Cambridge suggested that people with undetected coeliac disease had a beneficial risk profile for cardiovascular disease (less overweight, lower cholesterol levels). There is limited evidence that screen-detected cases benefit from a diagnosis in terms of morbidity and mortality; hence, population-level screening is not presently thought to be beneficial.

In the United Kingdom, the National Institute for Health and Clinical Excellence (NICE) recommends screening for coeliac disease in patients with newly diagnosed chronic fatigue syndrome  and irritable bowel syndrome, as well as in type 1 diabetics, especially those with insufficient weight gain or unexplained weight loss. It is also recommended in autoimmune thyroid disease, dermatitis herpetiformis, and in the first-degree relatives of those with confirmed coeliac disease.

There is a large number of scenarios where testing for coeliac disease may be offered given previously described associations, such as the conditions mentioned above in “miscelaneous”.

Treatment:-
General: Although there is currently no cure for celiac disease, the condition can be managed with diet. Symptoms will subside within several weeks and patients will be able to absorb food normally once they avoid eating gluten. However, it may take several months in children and two to three years in elderly patients for the intestine to fully recover.

Support: Healthcare providers may recommend a dietitian or nutritionist who can help a patient plan an appropriate gluten-free diet. These professionals can also help patients determine whether or not supplementation with vitamins and minerals is necessary.

Diet:
Gluten-free diet
At present, the only effective treatment is a life-long gluten-free diet. No medication exists that will prevent damage or prevent the body from attacking the gut when gluten is present. Strict adherence to the diet allows the intestines to heal, leading to resolution of all symptoms in most cases and, depending on how soon the diet is begun, can also eliminate the heightened risk of osteoporosis and intestinal cancer. Dietician input is generally requested to ensure the patient is aware which foods contain gluten, which foods are safe, and how to have a balanced diet despite the limitations. In many countries, gluten-free products are available on prescription and may be reimbursed by health insurance plans.

The diet can be cumbersome; failure to comply with the diet may cause relapse. The term gluten-free is generally used to indicate a supposed harmless level of gluten rather than a complete absence. The exact level at which gluten is harmless is uncertain and controversial. A recent systematic review tentatively concluded that consumption of less than 10 mg of gluten per day is unlikely to cause histological abnormalities, although it noted that few reliable studies had been done. Regulation of the label gluten-free varies widely by country. For example, in the United States, the term gluten-free is not yet regulated. The current international Codex Alimentarius standard, established in 1981, allows for 50 mg N/100 g on dry matter, although a proposal for a revised standard of 20 ppm in naturally gluten-free products and 200 ppm in products rendered gluten-free has been accepted. Gluten-free products are usually more expensive and harder to find than common gluten-containing foods. Since ready-made products often contain traces of gluten, some coeliacs may find it necessary to cook from scratch.

Even while on a diet, health-related quality of life (HRQOL) may be lower in people with coeliac disease. Studies in the United States have found that quality of life becomes comparable to the general population after staying on the diet, while studies in Europe have found that quality of life remains lower, although the surveys are not quite the same. Men tend to report more improvement than women. Some have persisting digestive symptoms or dermatitis herpetiformis, mouth ulcers, osteoporosis and resultant fractures. Symptoms suggestive of irritable bowel syndrome may be present, and there is an increased rate of anxiety, fatigue, dyspepsia and musculoskeletal pain.

Everyone is different, but many people with coeliac disease also have one or more additional food allergies or food intolerances, which may include milk protein (casein), corn (maize), soy, amines, or salicylates.

What if you eat gluten?
If you accidentally eat a product that contains gluten, you may experience abdominal pain and diarrhea. Some people experience no signs or symptoms after eating gluten, but this doesn’t mean it’s not hurting them. Even trace amounts of gluten in your diet can be damaging, whether or not they cause signs or symptoms.

Most people with celiac disease who follow a gluten-free diet have a complete recovery. Rarely, people with severely damaged small intestines don’t improve with a gluten-free diet. When diet isn’t effective, treatment often includes medications to help control intestinal inflammation and other conditions resulting from malabsorption.

Because celiac disease can lead to many complications, people who don’t respond to dietary changes need frequent monitoring for other health conditions.

Lifestyle and home remedies:-

Following a gluten-free diet may leave you angry and frustrated, understandably so. But with time, patience and a little creativity, you’ll find there are many foods that you can still eat and enjoy. Following are some tips to help you on your way to a safe and healthy diet.

Read food labels
Food labels are your lifeline to better health. Always read the food label before you purchase any product. Some foods that may appear acceptable, such as rice or corn cereals, may contain gluten. What’s more, a manufacturer may change a product’s ingredients at any time. A food that was once gluten-free no longer may be. Unless you read the label every time you shop, you won’t know this.

Call the manufacturer
If you can’t tell by the label if a food contains gluten, don’t eat it until you check with the product’s manufacturer. Some support groups produce a gluten-free shopper’s guide that can save you time at the market, although it may not be as current as that obtained from the manufacturer.

Don’t be afraid to eat out

Though preparing your own meals is the easiest way to monitor your diet, this doesn’t mean you can’t eat out. For an enjoyable dining experience, remember the following advice:

*Select places that specialize in the kinds of foods you can eat. You may want to call the restaurant in advance and discuss the menu options and your dietary needs.
*Be a repeat customer. Visit the same restaurants so that you become familiar with their menus and the personnel get to know your needs.
*Seek and share ideas. Ask members of your support group for suggestions on restaurants that serve gluten-free food. If there are enough gluten-sensitive people in your community, it’s likely that restaurant owners will try to satisfy your needs. Continue to share with the support group the names of any restaurants that add gluten-free foods to their menus.
*Follow the same practices you do at home. Select simply prepared or fresh foods and avoid all breaded or batter-coated foods, gravies and other foods with obvious or questionable ingredients.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://en.wikipedia.org/wiki/Coeliac_disease
http://www.mayoclinic.com/health/celiac-disease/DS00319
http://www.righthealth.com/topic/Celiac_Disease_Symptoms/overview/NaturalStandard20?fdid=NaturalStandard_5ba0efa8e0040c39deb1cd99a1446453&section=Full_Article

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