Celiac disease: Difference between revisions

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==Background<ref name="NEJM Celiac">Fasano A, Catassi C. Celiac Disease. New England Journal of Medicine. 2012;367(25):2419-2426. doi:10.1056/nejmcp1113994.</ref><ref name="Path">Parzanese I. Celiac disease: From pathophysiology to treatment. World Journal of Gastrointestinal Pathophysiology. 2017;8(2):27-38. doi:10.4291/wjgp.v8.i2.27.</ref><ref name="Complications">Freeman HJ. Adult Celiac Disease and Its Malignant Complications. Gut and Liver. 2009;3(4):237-246. doi:10.5009/gnl.2009.3.4.237.</ref>==
==Background<ref name="NEJM Celiac">Fasano A, Catassi C. Celiac Disease. New England Journal of Medicine. 2012;367(25):2419-2426. doi:10.1056/nejmcp1113994.</ref><ref name="Path">Parzanese I. Celiac disease: From pathophysiology to treatment. World Journal of Gastrointestinal Pathophysiology. 2017;8(2):27-38. doi:10.4291/wjgp.v8.i2.27.</ref><ref name="Complications">Freeman HJ. Adult Celiac Disease and Its Malignant Complications. Gut and Liver. 2009;3(4):237-246. doi:10.5009/gnl.2009.3.4.237.</ref>== <!--T:1-->


<!--T:2-->
[[File:Coeliac Disease.png|thumb|Schematic of the Marsh classification of upper jejunal pathology in coeliac disease.]]
[[File:Coeliac Disease.png|thumb|Schematic of the Marsh classification of upper jejunal pathology in coeliac disease.]]
*Also known as “Celiac Sprue”
*Also known as “Celiac Sprue”
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===Pathophysiology<ref name="NEJM Celiac"></ref><ref name="Path"></ref>===
===Pathophysiology<ref name="NEJM Celiac"></ref><ref name="Path"></ref>=== <!--T:3-->


<!--T:4-->
*Gluten triggers an immune mediated systemic disorder
*Gluten triggers an immune mediated systemic disorder
*Can be weeks to years between gluten exposure and onset of symptoms
*Can be weeks to years between gluten exposure and onset of symptoms
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==Clinical Features==
==Clinical Features== <!--T:5-->




===Classic Features===
===Classic Features=== <!--T:6-->


<!--T:7-->
*Classically presents around 6 to 18 months of age when foods containing gluten are introduced but can occur anytime
*Classically presents around 6 to 18 months of age when foods containing gluten are introduced but can occur anytime
*Chronic [[Special:MyLanguage/diarrhea|diarrhea]]
*Chronic [[Special:MyLanguage/diarrhea|diarrhea]]
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===Atypical Features===
===Atypical Features=== <!--T:8-->


<!--T:9-->
*Few or no gastrointestinal symptoms
*Few or no gastrointestinal symptoms
*Older children or adults
*Older children or adults
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===Silent or Asymptomatic From===
===Silent or Asymptomatic From=== <!--T:10-->


<!--T:11-->
*Patients have no symptoms
*Patients have no symptoms
*Diagnosed based on serology of histology
*Diagnosed based on serology of histology
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===Refractory Form===
===Refractory Form=== <!--T:12-->


<!--T:13-->
*Persistent or recurrent symptoms or villous atrophy despite gluten free diet for 12 months
*Persistent or recurrent symptoms or villous atrophy despite gluten free diet for 12 months




===Celiac Crisis<ref name="Crisis">Jamma S, Rubio–Tapia A, Kelly CP, et al. Celiac Crisis Is a Rare but Serious Complication of Celiac Disease in Adults. Clinical Gastroenterology and Hepatology. 2010;8(7):587-590. doi:10.1016/j.cgh.2010.04.009.</ref>===
===Celiac Crisis<ref name="Crisis">Jamma S, Rubio–Tapia A, Kelly CP, et al. Celiac Crisis Is a Rare but Serious Complication of Celiac Disease in Adults. Clinical Gastroenterology and Hepatology. 2010;8(7):587-590. doi:10.1016/j.cgh.2010.04.009.</ref>=== <!--T:14-->


<!--T:15-->
*Life threatening
*Life threatening
*Often observed after a general immune stimulus such as surgery or infection
*Often observed after a general immune stimulus such as surgery or infection
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===Complications from untreated disease<ref name="NEJM Celiac"></ref><ref name="Path"></ref><ref name="Complications"></ref>===
===Complications from untreated disease<ref name="NEJM Celiac"></ref><ref name="Path"></ref><ref name="Complications"></ref>=== <!--T:16-->


<!--T:17-->
*Osteoporosis
*Osteoporosis
*Impaired splenic function
*Impaired splenic function
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==Differential Diagnosis==
==Differential Diagnosis== <!--T:18-->


<!--T:19-->
*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
* Chronic diarrhea
* Chronic diarrhea
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==Evaluation<ref name="NEJM Celiac"></ref>==
==Evaluation<ref name="NEJM Celiac"></ref>== <!--T:20-->


<!--T:21-->
[[File:Celiac endo.png|thumb|Endoscopic still of duodenum of a person with celiac disease showing scalloping of folds and "cracked-mud" appearance to mucosa.]]
[[File:Celiac endo.png|thumb|Endoscopic still of duodenum of a person with celiac disease showing scalloping of folds and "cracked-mud" appearance to mucosa.]]
*Rule out emergent causes of [[Special:MyLanguage/abdominal pain|abdominal pain]] such as [[Special:MyLanguage/appendicitis|appendicitis]], [[Special:MyLanguage/small bowel obstruction|small bowel obstruction]], [[Special:MyLanguage/bowel perforation|bowel perforation]], etc.
*Rule out emergent causes of [[Special:MyLanguage/abdominal pain|abdominal pain]] such as [[Special:MyLanguage/appendicitis|appendicitis]], [[Special:MyLanguage/small bowel obstruction|small bowel obstruction]], [[Special:MyLanguage/bowel perforation|bowel perforation]], etc.
*Depending on presentation, chemistry panel and/or abdominal imaging may be appropriate in the emergency department setting
*Depending on presentation, chemistry panel and/or abdominal imaging may be appropriate in the emergency department setting


<!--T:22-->
{| {{table}}
{| {{table}}
| align="center" style="background:#f0f0f0;"|'''Test'''
| align="center" style="background:#f0f0f0;"|'''Test'''
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===Celiac Crisis<ref name="Crisis"></ref>===
===Celiac Crisis<ref name="Crisis"></ref>=== <!--T:23-->


<!--T:24-->
*History and physical
*History and physical
*Assessment of volume status
*Assessment of volume status
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*Lactic acid
*Lactic acid


<!--T:25-->
{| {{table}}
{| {{table}}
| align="center" style="background:#f0f0f0;"|'''Signs of severe dehydration including: hemodynamic instability and/or orthostatic changes'''
| align="center" style="background:#f0f0f0;"|'''Signs of severe dehydration including: hemodynamic instability and/or orthostatic changes'''
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==Management==
==Management== <!--T:26-->




===Uncomplicated Celiac disease===
===Uncomplicated Celiac disease=== <!--T:27-->


<!--T:28-->
*Avoid wheat, rye, barley which all contain gluten
*Avoid wheat, rye, barley which all contain gluten
*Possible benefit to avoiding large amounts of Oats
*Possible benefit to avoiding large amounts of Oats
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===Celiac Crisis<ref name="Crisis"></ref>===
===Celiac Crisis<ref name="Crisis"></ref>=== <!--T:29-->


<!--T:30-->
*[[Special:MyLanguage/Fluid resuscitation|Fluid resuscitation]]
*[[Special:MyLanguage/Fluid resuscitation|Fluid resuscitation]]
*Oral or IV [[Special:MyLanguage/steroids|steroids]]
*Oral or IV [[Special:MyLanguage/steroids|steroids]]
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==Disposition==
==Disposition== <!--T:31-->




===Uncomplicated Celiac disease===
===Uncomplicated Celiac disease=== <!--T:32-->


<!--T:33-->
*Primary care follow up:
*Primary care follow up:
**Serology, histology, and likely EGD
**Serology, histology, and likely EGD
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===Celiac Crisis===
===Celiac Crisis=== <!--T:34-->


<!--T:35-->
*Hospital admission
*Hospital admission




==See Also==
==See Also== <!--T:36-->


<!--T:37-->
*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
*[[Special:MyLanguage/Irritable bowel syndrome|Irritable bowel syndrome]]
*[[Special:MyLanguage/Irritable bowel syndrome|Irritable bowel syndrome]]
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==References==
==References== <!--T:38-->


<!--T:39-->
<references/>
<references/>
[[Category:GI]]
[[Category:GI]]
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Latest revision as of 12:29, 7 January 2026

Other languages:

Background[1][2][3]

Schematic of the Marsh classification of upper jejunal pathology in coeliac disease.
  • Also known as “Celiac Sprue”
  • Affects 0.6 to 1.0% of worldwide population
  • Occurs in industrialized countries and developing countries among all ethnicities and races
  • Occurs in children and adults
  • 1.5 to 2 times more prevalent in females than in males
  • Gluten is found in wheat, rye, and barley
  • Associated with strong family history, Type 1 DM, autoimmune thyroid disorders along with other autoimmune disorders, Turner’s syndrome, and Down syndrome
  • Rotavirus infection in children increases risk of developing disease
  • Breastfeeding reported to reduce risk by 50%
  • MHC class II proteins HLA-DQ2 (90%) and HLA-DQ8 are expressed in almost all of patients affected by Celiac disease


Pathophysiology[1][2]

  • Gluten triggers an immune mediated systemic disorder
  • Can be weeks to years between gluten exposure and onset of symptoms
  • Gluten is mainly composed of prolamine and glutanin
    • Prolamines (such as Gliadin found in wheat) interact with intestinal cells to cause breakdown of the inter-enterocyte tight junctions
  • Proposed that HLA-DQ2 and HLA-DQ8 present prolamines to antigen-specific CD4+ T lymphocytes in lamina propria of the small bowel
  • Once activated, CD4+ T cells release pro-inflammatory cytokines and activate T-helper cells
  • Proinflammatory cytokines such as IL-15 stimulate CD8+ T-lymphocytes
  • T-helper type 1 cells release IFN-gamma
  • T-helper type 2 cells promote conversion of B cells to plasma cells that produce anti-gliadin and anti-tissue-transglutaminase antibodies that are used in serologic testing
  • This process leads to celiac enteropathy with histological features such as decreased enterocyte height, crypt hyperplasia, villous atrophy, and increased intraepithelial T lymphocytes


Clinical Features

Classic Features

  • Classically presents around 6 to 18 months of age when foods containing gluten are introduced but can occur anytime
  • Chronic diarrhea
  • Weight loss
  • Abdominal distension
  • Iron deficiency with or without anemia
  • Aphthous stomatitis
  • Short stature
  • High aminotransferase levels
  • Chronic fatigue
  • Reduced bone mineral density
  • Vitamin D deficiency


Atypical Features


Silent or Asymptomatic From

  • Patients have no symptoms
  • Diagnosed based on serology of histology
  • Found in patients with strong family history of disease, autoimmune disorders, chromosomal disorders (Down syndrome)


Refractory Form

  • Persistent or recurrent symptoms or villous atrophy despite gluten free diet for 12 months


Celiac Crisis[4]

  • Life threatening
  • Often observed after a general immune stimulus such as surgery or infection
  • Mostly observed in children
  • Severe diarrhea
  • Hypoproteinemia
  • Metabolic and electrolyte disturbances


Complications from untreated disease[1][2][3]

  • Osteoporosis
  • Impaired splenic function
  • Infertility
  • Recurrent abortion
  • Ulcerative jejunoileitis
  • Enteropathy-associated intestinal T-cell lymphoma
  • Adenocarcinoma of the jejunum
  • Non-Hodgkin lymphoma
  • Vitamin deficiencies


Differential Diagnosis


Evaluation[1]

Endoscopic still of duodenum of a person with celiac disease showing scalloping of folds and "cracked-mud" appearance to mucosa.
Test Sensitivity Specificity Comments
IgA anti-tTG antibodies >95 >95 Screening test
IgG anti-tTG antibodies Variable Variable Screening test in patients with IgA deficiency
IgA antendomysial antibodies >90 98.2 Useful if diagnosis unclear
IgG DGP >90 >90 IgA deficiency and young patients
HLA DQ2 or HLA-DQ8 91 45 High NPV


Celiac Crisis[4]

  • History and physical
  • Assessment of volume status
  • CBC to look for evidence of iron deficiency anemia
  • CMP
  • VBG
  • Lactic acid
Signs of severe dehydration including: hemodynamic instability and/or orthostatic changes
Neurologic dysfunction
Renal dysfunction: creatinine >2.0 g/dL
Metabolic acidosis: pH <7.35
Hypoproteinemia (Albumin < 3.0 g/dL)
Abnormal electrolytes including: hyper/hyponatremia, hypocalcemia, hypokalemia or hypomagnesemia
Weight loss > 10 lbs


Management

Uncomplicated Celiac disease

  • Avoid wheat, rye, barley which all contain gluten
  • Possible benefit to avoiding large amounts of Oats
  • IV fluids if necessary


Celiac Crisis[4]


Disposition

Uncomplicated Celiac disease

  • Primary care follow up:
    • Serology, histology, and likely EGD
    • consultation with dietitian
    • treatment of nutritional deficiencies
    • referral to specialists
    • serologic monitoring for recurrence


Celiac Crisis

  • Hospital admission


See Also


References

  1. ↑ 1.0 1.1 1.2 1.3 Fasano A, Catassi C. Celiac Disease. New England Journal of Medicine. 2012;367(25):2419-2426. doi:10.1056/nejmcp1113994.
  2. ↑ 2.0 2.1 2.2 Parzanese I. Celiac disease: From pathophysiology to treatment. World Journal of Gastrointestinal Pathophysiology. 2017;8(2):27-38. doi:10.4291/wjgp.v8.i2.27.
  3. ↑ 3.0 3.1 Freeman HJ. Adult Celiac Disease and Its Malignant Complications. Gut and Liver. 2009;3(4):237-246. doi:10.5009/gnl.2009.3.4.237.
  4. ↑ 4.0 4.1 4.2 Jamma S, Rubio–Tapia A, Kelly CP, et al. Celiac Crisis Is a Rare but Serious Complication of Celiac Disease in Adults. Clinical Gastroenterology and Hepatology. 2010;8(7):587-590. doi:10.1016/j.cgh.2010.04.009.
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