Celiac disease

Changed by Yaïr Glick, 7 Nov 2016

Updates to Article Attributes

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Coeliac disease, also known as non-tropical sprue, is a T-cell mediated autoimmune chronic gluten intolerance condition characterised by loss of villi in the proximal small bowel and gastrointestinal malabsorption (sprue).

It should always be considered as a possible underlying aetiology in cases of iron deficiency anaemia of uncertain cause.

Epidemiology

Coeliac disease is relatively common in Caucasians, 1 in 200, but it is extremely rare in Asian orand black people. There are two peaks of presentation, a small number of patients present early in childhood and the second more common, larger group of patients presents at 3rd and 4th decades. 

Clinical presentation

Many patients have a paucity of symptoms with no GI upset. However, abdominal pain is considered the most common symptomssymptom. Other manifestations include:

  • iron deficiency anaemia and guaiac-positive stools
  • diarrhoea
  • constipation
  • malabsorption, including fat soluble-soluble vitamins
  • weight loss

Pathology

Coeliac disease is a chronic autoimmune disease induced in genetically susceptible individuals after ingestion of gluten. Small bowel mucosa is primarily affected (submucosa, muscularis and serosa remain normalunaffected) causing, resulting in progressive degrees of villous inflammation and destruction. The disease tends to start in the duodenum and extends into the ilium, resulting in induction crypt hyperplasia. Loss of villi, which absorbsabsorb fluid, and hypertrophy of crypts, which produce fluid, result in a fluid excess in the small bowel lumen 8.  

The villous atrophy that occurs within the bowel also results in malabsorption of iron, folic acid, calcium and fat-soluble vitamin resultingvitamins manifesting in a variety of signs, some of which may be non-specific.

The gold standard diagnostic test is a duodenal biopsy taken at endoscopy.

Histology
  • total villous loss, initially blunting progressing to flattened mucosa
  • hyperplasia of the crypts 
  • epithelial infiltration with T-cell lymphocytes
Markers

Additionally, serum antibodies may be raised:

  • anti-tissue transglutaminase antibody (anti-tTG), IgA
  • deamidated gliadin peptide (DGP) antibodies, IgA
  • anti-endomysial antibodies (EMA), IgA class
  • anti-reticulin antibodies (ARA), IgA class

Quantitative immunoglobulin A (IgA): measures the total level of IgA in the blood to determine if someone is deficient in the IgA class of antibodies. The IgG class of anti-tTG may be ordered for people who have a deficiency of IgA.

Associations

Radiographic features

Fluoroscopy

Features of small bowel barium studies are not sensitive enough for confident diagnosis, but somethe following changes may be seen:

CT enteroclysis

Features present on CT enteroclysis may include 3,6:

  • reversed jejunoileal fold pattern: thought to have the highest specificity is considered the most discriminating independent variable for the diagnosis of uncomplicated coeliac disease
  • ileal fold thickening
  • vascular engorgement
  • prominent mesenteric lymph nodes may cavitate with a fluid fat level
  • submucosal fat deposition in long standing cases
  • other adjunctive features

Treatment and prognosis

Complications
  • -<p><strong>Coeliac disease</strong>, also known as <strong>non-tropical sprue</strong>, is a T-cell mediated autoimmune chronic gluten intolerance condition characterised by loss of villi in the proximal small bowel and gastrointestinal malabsorption (<a href="/articles/sprue">sprue</a>).</p><p>It should always be considered as a possible underlying aetiology in cases of <a href="/articles/iron-deficiency-anaemia">iron deficiency anaemia</a> of uncertain cause.</p><h4>Epidemiology</h4><p>Coeliac disease is relatively common in Caucasians, 1 in 200, but it is extremely rare in Asian or black people. There are two peaks of presentation, a small number of patients present early in childhood and the second more common group of patients presents at 3<sup>rd </sup>and 4<sup>th </sup>decades. </p><h4>Clinical presentation</h4><p>Many patients have a paucity of symptoms with no GI upset. However, abdominal pain is considered the most common symptoms. Other manifestations include:</p><ul>
  • +<p><strong>Coeliac disease</strong>, also known as <strong>non-tropical sprue</strong>, is a T-cell mediated autoimmune chronic gluten intolerance condition characterised by loss of villi in the proximal small bowel and gastrointestinal malabsorption (<a href="/articles/sprue">sprue</a>).</p><p>It should always be considered as a possible underlying aetiology in cases of <a href="/articles/iron-deficiency-anaemia">iron deficiency anaemia</a> of uncertain cause.</p><h4>Epidemiology</h4><p>Coeliac disease is relatively common in Caucasians, 1 in 200, but it is extremely rare in Asian and black people. There are two peaks of presentation, a small number of patients present early in childhood and the second, larger group of patients presents at 3<sup>rd </sup>and 4<sup>th </sup>decades. </p><h4>Clinical presentation</h4><p>Many patients have a paucity of symptoms with no GI upset. However, abdominal pain is considered the most common symptom. Other manifestations include:</p><ul>
  • -<li>malabsorption including fat soluble vitamins</li>
  • +<li>malabsorption, including fat-soluble vitamins</li>
  • -</ul><h4>Pathology</h4><p>Coeliac disease is a chronic autoimmune disease induced in genetically susceptible individuals after ingestion of gluten. <a href="/articles/small-bowel">Small bowel</a> mucosa is primarily affected (submucosa, muscularis and serosa remain normal) causing progressive degrees of villous inflammation and destruction. The disease tends to start in the duodenum and extends into ilium resulting in induction crypt hyperplasia. Loss of villi, which absorbs fluid, and hypertrophy of crypts, which produce fluid, result in a fluid excess in the small bowel lumen <sup>8</sup>.  </p><p>The villous atrophy that occurs within the bowel also results in <a href="/articles/malabsorption">malabsorption</a> of iron, folic acid, calcium and fat-soluble vitamin resulting in a variety of signs, some of which may be non-specific.</p><p>The gold standard diagnostic test is a duodenal biopsy taken at endoscopy.</p><h5>Histology</h5><ul>
  • +</ul><h4>Pathology</h4><p>Coeliac disease is a chronic autoimmune disease induced in genetically susceptible individuals after ingestion of gluten. <a href="/articles/small-bowel">Small bowel</a> mucosa is primarily affected (submucosa, muscularis and serosa remain unaffected), resulting in progressive degrees of villous inflammation and destruction. The disease tends to start in the duodenum and extends into the ilium, resulting in induction crypt hyperplasia. Loss of villi, which absorb fluid, and hypertrophy of crypts, which produce fluid, result in a fluid excess in the small bowel lumen <sup>8</sup>.  </p><p>The villous atrophy that occurs within the bowel also results in <a href="/articles/malabsorption">malabsorption</a> of iron, folic acid, calcium and fat-soluble vitamins manifesting in a variety of signs, some of which may be non-specific.</p><p>The gold standard diagnostic test is a duodenal biopsy taken at endoscopy.</p><h5>Histology</h5><ul>
  • -<a href="/articles/small-bowel-lymphoma-1">small bowel lymphoma</a>, in particular, enteropathy-associated T cell lymphoma but also other <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphomas</a> <sup>11</sup>
  • +<a href="/articles/small-bowel-lymphoma-1">small bowel lymphoma</a>, in particular, enteropathy-associated T cell lymphoma, but also other <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphomas</a> <sup>11</sup>
  • -</ul><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>Features of small bowel barium studies are not sensitive enough for confident diagnosis, but some changes may be seen:</p><ul>
  • +</ul><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>Features of small bowel barium studies are not sensitive enough for confident diagnosis, but the following changes may be seen:</p><ul>
  • -<a href="/articles/cavitatory-mesenteric-lymph-node-syndrome">cavitatory lymph node syndrome</a><a href="/articles/cavitating-mesenteric-lymphadenopathy-syndrome"> </a><sup>1-2</sup><ul><li>
  • +<a href="/articles/cavitatory-mesenteric-lymph-node-syndrome">cavitary lymph node syndrome</a><a href="/articles/cavitating-mesenteric-lymphadenopathy-syndrome"> </a><sup>1-2</sup><ul><li>

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