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Sprue (Celiac Disease)
Celiac disease is a digestive disease that damages the small intestine and
interferes with absorption of nutrients from food. People who have celiac
disease cannot tolerate a protein called gluten, which is found in wheat, rye,
barley, and possibly oats. When people with celiac disease eat foods containing
gluten, their immune system responds by damaging the small intestine.
Specifically, tiny fingerlike protrusions, called villi, on the lining of the
small intestine are lost. Nutrients from food are absorbed into the bloodstream
through these villi. Without villi, a person becomes malnourished--regardless of
the quantity of food eaten. Because the body's own immune system causes
the damage, celiac disease is considered an autoimmune disorder. However, it is
also classified as a disease of malabsorption because nutrients are not
absorbed. Celiac disease is also known as celiac sprue, nontropical sprue, and
Celiac disease is a genetic disease, meaning that it runs in families.
Sometimes the disease is triggered--or becomes active for the first time--after
surgery, pregnancy, childbirth, viral infection, or severe emotional stress.
Celiac disease affects people differently. Some people develop symptoms as
children, others as adults. One factor thought to play a role in when and how
celiac appears is whether and how long a person was breastfed--the longer one
was breastfed, the later symptoms of celiac disease appear, and the more
atypical the symptoms. Other factors include the age at which one began eating
foods containing gluten and how much gluten is eaten. Symptoms may or may
not occur in the digestive system. For example, one person might have diarrhea
and abdominal pain, while another person has irritability or depression. In
fact, irritability is one of the most common symptoms in children.
|Villi on the
lining of the small intestine help
Symptoms of celiac disease may include one or more of the following:
- Recurring abdominal bloating and pain.
- Chronic diarrhea.
- Weight loss.
- Pale, foul-smelling stool.
- Unexplained anemia (low count of red blood cells).
- Bone pain.
- Behavior changes.
- Muscle cramps.
- Delayed growth.
- Failure to thrive in infants.
- Pain in the joints.
- Tingling numbness in the legs (from nerve damage).
- Pale sores inside the mouth, called aphthus ulcers.
- Painful skin rash, called dermatitis herpetiformis.
- Tooth discoloration or loss of enamel.
- Missed menstrual periods (often because of excessive weight loss).
Anemia, delayed growth, and weight loss are signs of malnutrition--not
getting enough nutrients. Malnutrition is a serious problem for anyone, but
particularly for children because they need adequate nutrition to develop
properly. Some people with celiac disease may not have symptoms. The
undamaged part of their small intestine is able to absorb enough nutrients to
prevent symptoms. However, people without symptoms are still at risk for the
complications of celiac disease. Diagnosing celiac disease can be
difficult because some of its symptoms are similar to those of other diseases,
including irritable bowel syndrome, Crohn's disease, ulcerative colitis,
diverticulosis, intestinal infections, chronic fatigue syndrome, and depression.
Recently, researchers discovered that people with celiac disease have higher
than normal levels of certain antibodies in their blood. Antibodies are produced
by the immune system in response to substances that the body perceives to be
threatening. To diagnose celiac disease, physicians test blood to measure levels
of antibodies to gluten. These antibodies are antigliadin, anti-endomysium, and
If the tests and symptoms suggest celiac disease, the physician may remove a
tiny piece of tissue from the small intestine to check for damage to the villi.
This is done in a procedure called a biopsy: the physician eases a long, thin
tube called an endoscope through the mouth and stomach into the small intestine,
and then takes a sample of tissue using instruments passed through the endoscope.
Biopsy of the small intestine is the best way to diagnose celiac disease.
Screening for celiac disease involves testing asymptomatic people for the
antibodies to gluten. Americans are not routinely screened for celiac disease.
However, because celiac disease is hereditary, family members--particularly
first-degree relatives--of people who have been diagnosed may need to be tested
for the disease. About 10 percent of an affected person's first-degree relatives
(parents, siblings, or children) will also have the disease. The longer a person
goes undiagnosed and untreated, the greater the chance of developing
malnutrition and other complications. The only treatment for celiac
disease is to follow a gluten-free diet--that is, to avoid all foods that
contain gluten. For most people, following this diet will stop symptoms, heal
existing intestinal damage, and prevent further damage. Improvements begin
within days of starting the diet, and the small intestine is usually completely
healed--meaning the villi are intact and working--in 3 to 6 months. (It may take
up to 2 years for older adults.)
The gluten-free diet is a lifetime requirement. Eating any gluten, no matter
how small an amount, can damage the intestine. This is true for anyone with the
disease, including people who do not have noticeable symptoms. Depending on a
person's age at diagnosis, some problems, such as delayed growth and tooth
discoloration, may not improve. A small percentage of people with celiac
disease do not improve on the gluten-free diet. These people often have severely
damaged intestines that cannot heal even after they eliminate gluten from their
diets. Because their intestines are not absorbing enough nutrients, they may
need to receive intravenous nutrition supplements. Drug treatments are being
evaluated for unresponsive celiac disease. These patients may need to be
evaluated for complications of the disease. If a person responds to the
gluten-free diet, the physician will know for certain that the diagnosis of
celiac disease is correct.
The Gluten-Free Diet
A gluten-free diet means avoiding all foods that contain wheat (including spelt,
triticale, and kamut), rye, barley, and possibly oats--in other words, most
grain, pasta, cereal, and many processed foods. Despite these restrictions,
people with celiac disease can eat a well-balanced diet with a variety of foods,
including bread and pasta. For example, instead of wheat flour, people can use
potato, rice, soy, or bean flour. Or, they can buy gluten-free bread, pasta, and
other products from special food companies.
Whether people with celiac disease should avoid oats is controversial because
some people have been able to eat oats without having a reaction. Scientists are
doing studies to find out whether people with celiac disease can tolerate oats.
Until the studies are complete, people with celiac disease should follow their
physician or dietitian's advice about eating oats. Plain meat, fish, rice,
fruits, and vegetables do not contain gluten, so people with celiac disease can
eat as much of these foods as they like.
The gluten-free diet is complicated. It requires a completely new approach to
eating that affects a person's entire life. People with celiac disease have to
be extremely careful about what they buy for lunch at school or work, eat at
cocktail parties, or grab from the refrigerator for a midnight snack. Eating out
can be a challenge as the person with celiac disease learns to scrutinize the
menu for foods with gluten and question the waiter or chef about possible hidden
sources of gluten. However, with practice, screening for gluten becomes second
nature and people learn to recognize which foods are safe and which are off
limits. A dietitian, a health care professional who specializes in food
and nutrition, can help people learn about their new diet. Also, support groups
are particularly helpful for newly diagnosed people and their families as they
learn to adjust to a new way of life.
Long term complications of Celiac disease are well known. Damage to the
small intestine and the resulting problems with nutrient absorption put a person
with celiac disease at risk for several diseases and health problems.
Celiac disease is the most common genetic disease in Europe. In Italy about 1 in
250 people and in Ireland about 1 in 300 people have celiac disease. It is
rarely diagnosed in African, Chinese, and Japanese people. An estimated 1
in 4,700 Americans have been diagnosed with celiac disease. Some researchers
question how celiac disease could be so uncommon in the United States since it
is hereditary and many Americans descend from European ethnic groups in whom the
disease is common. A recent study in which random blood samples from the Red
Cross were tested for celiac disease suggests that as many as 1 in every 250
Americans may have it. More research is needed to find out the true
prevalence of celiac disease among Americans. Celiac disease could be
underdiagnosed in the United States for a number of reasons:
- Lymphoma and adenocarcinoma are types of cancer that can develop in
- Osteoporosis is a condition in which the bones become weak,
brittle, and prone to breaking. Poor calcium absorption is a contributing
factor to osteoporosis.
- Miscarriage and congenital malformation of the baby, such as neural
tube defects, are risks for untreated pregnant women with celiac disease
because of malabsorption of nutrients.
- Short stature results when childhood celiac disease prevents
nutrient absorption during the years when nutrition is critical to a child's
normal growth and development. Children who are diagnosed and treated before
their growth stops may have a catch-up period.
- Seizures, or convulsions, result from inadequate absorption of
folic acid. Lack of folic acid causes calcium deposits, called
calcifications, to form in the brain, which in turn cause seizures.
- Celiac symptoms can be attributed to other problems.
- Many doctors are not knowledgeable about the disease
- Only a handful of U.S. laboratories are experienced and skilled in testing
for celiac disease.
People with celiac disease tend to have other autoimmune diseases as well.
The connection between celiac and these diseases may be genetic. The
diseases include the following:
- Dermatitis herpetiformis.
- Thyroid disease.
- Systemic lupus erythematosus.
- Type 1 diabetes.
- Liver disease.
- Collagen vascular disease.
- Rheumatoid arthritis.
- Sjogren's syndrome.