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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>==

[[File:Coeliac Disease.png|thumb|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 [[Special:MyLanguage/DM|DM]], autoimmune [[Special:MyLanguage/thyroid disorder|thyroid disorder]]s along with other autoimmune disorders, Turner’s syndrome, and [[Special:MyLanguage/Down syndrome|Down syndrome]]
*[[Special:MyLanguage/Rotavirus|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<ref name="NEJM Celiac"></ref><ref name="Path"></ref>===

*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 [[Special:MyLanguage/diarrhea|diarrhea]]
*Weight loss
*Abdominal distension
*Iron deficiency with or without [[Special:MyLanguage/anemia|anemia]]
*[[Special:MyLanguage/Aphthous stomatitis|Aphthous stomatitis]]
*Short stature
*High aminotransferase levels
*Chronic fatigue
*Reduced bone mineral density
*Vitamin D deficiency

===Atypical Features===

*Few or no gastrointestinal symptoms
*Older children or adults
*[[Special:MyLanguage/Dermatitis herpetiformis|Dermatitis herpetiformis]]
*Gluten Ataxia

===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 ([[Special:MyLanguage/Down syndrome|Down syndrome]])

===Refractory Form===

*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>===

*Life threatening
*Often observed after a general immune stimulus such as surgery or infection
*Mostly observed in children
*Severe diarrhea
*Hypoproteinemia
*Metabolic and [[Special:MyLanguage/electrolyte disturbance|electrolyte disturbance]]s

===Complications from untreated disease<ref name="NEJM Celiac"></ref><ref name="Path"></ref><ref name="Complications"></ref>===

*Osteoporosis
*Impaired splenic function
*Infertility
*Recurrent [[Special:MyLanguage/spontaneous abortion|abortion]]
*Ulcerative jejunoileitis
*Enteropathy-associated intestinal T-cell [[Special:MyLanguage/lymphoma|lymphoma]]
*Adenocarcinoma of the jejunum
*Non-Hodgkin lymphoma
*[[Special:MyLanguage/Vitamin deficiencies|Vitamin deficiencies]]

==Differential Diagnosis==

*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
* Chronic diarrhea
*[[Special:MyLanguage/Irritable bowel syndrome|Irritable bowel syndrome]]
*[[Special:MyLanguage/Small intestinal bacterial overgrowth|Small intestinal bacterial overgrowth]]
*Lactose intolerance
*[[Special:MyLanguage/Chronic pancreatitis|Chronic pancreatitis]]
*Microscopic colitis
*[[Special:MyLanguage/Inflammatory bowel disease|Inflammatory bowel disease]]
*Non-celiac gluten sensitivity (NCGS)
*Wheat allergy
*[[Special:MyLanguage/vitamin deficiencies|Vitamin or mineral deficiency]]

==Evaluation<ref name="NEJM Celiac"></ref>==

[[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.
*Depending on presentation, chemistry panel and/or abdominal imaging may be appropriate in the emergency department setting

{| {{table}}
| align="center" style="background:#f0f0f0;"|'''Test'''
| align="center" style="background:#f0f0f0;"|'''Sensitivity'''
| align="center" style="background:#f0f0f0;"|'''Specificity'''
| align="center" style="background:#f0f0f0;"|'''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<ref name="Crisis"></ref>===

*History and physical
*Assessment of volume status
*CBC to look for evidence of iron deficiency anemia
*CMP
*VBG
*Lactic acid

{| {{table}}
| align="center" style="background:#f0f0f0;"|'''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<ref name="Crisis"></ref>===

*[[Special:MyLanguage/Fluid resuscitation|Fluid resuscitation]]
*Oral or IV [[Special:MyLanguage/steroids|steroids]]
*Supplemental nutrition
*[[Special:MyLanguage/Electrolyte repletion|Electrolyte repletion]]
*Gluten free diet

==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==

*[[Special:MyLanguage/Acute diarrhea|Acute diarrhea]]
*[[Special:MyLanguage/Irritable bowel syndrome|Irritable bowel syndrome]]
*[[Special:MyLanguage/Inflammatory bowel disease|Inflammatory bowel disease]]

==References==

<references/>
[[Category:GI]]

Celiac disease/en - Revision history

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