Credit: wikipedia.org (Biopsy of small bowel showing coeliac disease manifested by blunting of villi, crypt hyperplasia, and lymphocyte infiltration of crypts) |
Coeliac disease, also spelled celiac disease, is an autoimmune disorder affecting primarily
the small intestine that occurs in people who are genetically predisposed. Classic
symptoms include gastrointestinal problems such as chronic diarrhoea,
abdominal distention, malabsorption, loss of appetite, and among children failure
to grow normally. This often begins between six months and two years of
age. Non-classic
symptoms are the most common, especially in people older than two years. There
may be mild or absent gastrointestinal symptoms, a wide number of symptoms
involving any part of the body, or no obvious symptoms. Coeliac
disease was first described in childhood; however,
it may develop at any age. It
is associated with other autoimmune diseases, such as diabetes mellitus type 1 and thyroiditis,
among others.
Coeliac disease is caused by a reaction to gluten, which are
various proteins found in wheat and in other grains such as barley, and rye. Moderate
quantities of oats, free of contamination with other gluten-containing grains,
are usually tolerated but
problems may depend on the type consumed. Upon
exposure to gluten, an abnormal immune response
may lead to the production of several different autoantibodies that
can affect a number of different organs. In
the small-bowel this causes an inflammatory
reaction and may produce shortening of the villi lining the small
intestine (villous atrophy). This
affects the absorption of nutrients, frequently leading to anaemia.
Diagnosis is typically made by a combination of blood
antibody tests and intestinal biopsies,
helped by specific genetic testing. Making
the diagnosis is not always straightforward. Frequently,
the autoantibodies in the blood are negative and
many people have only minor intestinal changes with normal villi. People
may have severe symptoms and be investigated for years before a diagnosis is
achieved. Increasingly,
the diagnosis is being made in people
without symptoms as a result of increased screening. While
the disease is caused by a permanent intolerance to wheat proteins, it is
usually classified as different from the other forms of wheat
allergy.
The only known effective treatment is a strict lifelong gluten-free
diet, which leads to recovery of the intestinal mucosa, improves symptoms,
and reduced risk of developing complications in most people. If
untreated it may result in cancers such as intestinal lymphoma and
a slight increased risk of early death. Rates
vary between different regions of the world, from as few as 1 in 300 to as many
as 1 in 40, with an average of between 1 in 100 and 1 in 170 people. In
developed countries, it is estimated that five out of six cases (83%) remain
undiagnosed, usually because of non-classic, minimal, or absent complaints. Coeliac
disease is slightly more common in women than in men. The
term "coeliac" is from the Greek κοιλιακός (koiliakós,
"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.
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