Celiac disease
Updates to Article Attributes
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 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 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 symptoms. Other manifestations include:
- iron deficiency anaemia and guaiac-positive stools
- diarrhoea
- constipation
- malabsorption including fat 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 normal) causing progressive degrees of villous inflammation and destruction. The disease tends to startsstart 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 8.
The villous atrophy that occurs within the bowel also results in malabsorption of iron, folic acid, calcium and fat soluble-soluble vitamin resulting 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
- idiopathic pulmonary haemosiderosis: as part of the Lane Hamilton syndrome 4
- dermatitis herpetiformis
- IgA deficiency
- cavitary lymph node syndrome
- small bowel lymphoma, in particular, enteropathy-associated T cell lymphoma but also other non-Hodgkin lymphomas 11
- Down syndrome (trisomy 21) 12
Radiographic features
Fluoroscopy
Features of small bowel barium studies are not sensitive enough for confident diagnosis, but some changes may be seen:
- small intestinal dilatation due to excess fluid
- dilution of contrast
- multiple non-obstructing
intussusceptionintussusceptions - jejunoileal fold pattern reversal
- moulage sign
- mosaic pattern
- flocculation
- segmentation
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
- increased risk of malignant conditions such as small bowel lymphoma(mainly T cell type) and small bowel adenocarcinoma
-
ulcerative
jejuno ileitisjejunoileitis 1 - increase risk of development of carcinoma of the oesophagus
- cavitatory lymph node syndrome1-2
-<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, 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 paucity of symptoms with no GI upset. However, abdominal pain is considered the most common symptoms. Other manifestations include:</p><ul>-<li>iron deficiency anaemia and guaiac-positive stools </li>- +<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>
- +<li>iron deficiency anaemia and guaiac-positive stools</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 starts in 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 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 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>
-<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>
-<li>multiple non-obstructing <a href="/articles/intussusception">intussusception</a>- +<li>multiple non-obstructing <a href="/articles/intussusception">intussusceptions</a>
-<li>prominent mesenteric lymph nodes, may cavitate with fluid fat level</li>- +<li>prominent mesenteric lymph nodes may cavitate with a fluid fat level</li>
-</ul><h4>Complications</h4><ul>-<li>increased risk of malignant conditions such as <a href="/articles/small-bowel-lymphoma-1">small bowel lymphoma</a><a href="/articles/small-bowel-lymphoma-"> </a>(mainly T cell type) and <a href="/articles/small-bowel-adenocarcinoma">small bowel adenocarcinoma</a>- +</ul><h4>Treatment and prognosis</h4><h5>Complications</h5><ul>
- +<li>increased risk of malignant conditions such as <a href="/articles/small-bowel-lymphoma-1">small bowel lymphoma</a> (mainly T cell type) and <a href="/articles/small-bowel-adenocarcinoma">small bowel adenocarcinoma</a>
-<a href="/articles/ulcerative-jejuno-ileitis-">ulcerative jejuno ileitis </a><sup>1</sup>- +<a href="/articles/ulcerative-jejuno-ileitis-">ulcerative jejunoileitis </a><sup>1</sup>
-<li>increase risk of development of <a href="/articles/oesophageal-carcinoma-1">carcinoma of oesophagus</a>- +<li>increase risk of development of <a href="/articles/oesophageal-carcinoma-1">carcinoma of the oesophagus</a>