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'''Celiac sprue''', also '''celiac disease''', is a common pathology that affects the [[duodenum]].  
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Celiac disease - high mag.jpg
| Width      =
| Caption    = Celiac disease. [[H&E stain]].
| Synonyms  = celiac disease
| Micro      = Intraepithelial lymphocytes +/- villous blunting
| Subtypes  =
| LMDDx      = [[Giardiasis]], [[Enteropathy-associated T-cell lymphoma]] ([[EATL]]), [[inflammatory bowel disease]], [[MALT lymphoma]], others
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[Duodenum]]
| Assdx      = [[dermatitis herpetiformis]], IgA deficiency, [[EATL]], [[duodenal adenocarcinoma]]
| Syndromes  =
| Clinicalhx = improves with gluten free diet
| Signs      = diarrhea
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  = TTG elevated (>10 U/mL)
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    = [[normal duodenum]]
| Tx        = gluten free diet
}}
'''Celiac sprue''', also '''celiac disease''' (abbreviated '''CD'''), is a common pathology that affects the [[duodenum]].
 
It should not be confused with ''tropical sprue''.


An introduction to gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.  It covers basic gastrointestinal histology.
An introduction to gastrointestinal pathology is in the ''[[gastrointestinal pathology]]'' article.  It covers basic gastrointestinal histology.


==Etiology==
==General==
*Autoimmune.
*Considered an autoimmune disease.
*The typical individual with celiac disease has a normal weight or is underweight.
**[[Obese]] and overweight individuals can have celiac disease.<ref name=pmid26892766>{{Cite journal  | last1 = Singh | first1 = I. | last2 = Agnihotri | first2 = A. | last3 = Sharma | first3 = A. | last4 = Verma | first4 = AK. | last5 = Das | first5 = P. | last6 = Thakur | first6 = B. | last7 = Sreenivas | first7 = V. | last8 = Gupta | first8 = SD. | last9 = Ahuja | first9 = V. | title = Patients with celiac disease may have normal weight or may even be overweight. | journal = Indian J Gastroenterol | volume =  | issue =  | pages =  | month = Feb | year = 2016 | doi = 10.1007/s12664-016-0620-9 | PMID = 26892766 }}</ref>


==Epidemiology==
===Epidemiology===
*Associated with:
Associated with:
**The skin condition ''[[dermatitis herpetiformis]]''.<ref>TN 2007 D22</ref>
*[[Dermatitis herpetiformis]] - skin condition.<ref name=Ref_TN2007_D22>{{Ref TN2007| D22}}</ref>
***Tx: dapsone.
**Tx: dapsone.
**IgA deficiency - 10-15X more common in celiac disease vs. healthy controls.<ref name=pmid12414763>{{Cite journal  | last1 = Kumar | first1 = V. | last2 = Jarzabek-Chorzelska | first2 = M. | last3 = Sulej | first3 = J. | last4 = Karnewska | first4 = K. | last5 = Farrell | first5 = T. | last6 = Jablonska | first6 = S. | title = Celiac disease and immunoglobulin a deficiency: how effective are the serological methods of diagnosis? | journal = Clin Diagn Lab Immunol | volume = 9 | issue = 6 | pages = 1295-300 | month = Nov | year = 2002 | doi =  | PMID = 12414763 }}</ref>
*IgA deficiency - 10-15X more common in celiac disease vs. healthy controls.<ref name=pmid12414763>{{Cite journal  | last1 = Kumar | first1 = V. | last2 = Jarzabek-Chorzelska | first2 = M. | last3 = Sulej | first3 = J. | last4 = Karnewska | first4 = K. | last5 = Farrell | first5 = T. | last6 = Jablonska | first6 = S. | title = Celiac disease and immunoglobulin a deficiency: how effective are the serological methods of diagnosis? | journal = Clin Diagn Lab Immunol | volume = 9 | issue = 6 | pages = 1295-300 | month = Nov | year = 2002 | doi =  | PMID = 12414763 }}</ref>
**Risk factor for ''gastrointestinal T cell lymphoma'' - known as: ''[[enteropathy-associated T cell lymphoma]]'' (EATL).
*Risk factor for ''gastrointestinal T cell lymphoma'' - known as: ''[[enteropathy-associated T cell lymphoma]]'' (EATL).
*[[IgA nephropathy]] is associated with an increased frequency of [[celiac disease]].<ref name=pmid19332868>{{Cite journal  | last1 = Smerud | first1 = HK. | last2 = Fellström | first2 = B. | last3 = Hällgren | first3 = R. | last4 = Osagie | first4 = S. | last5 = Venge | first5 = P. | last6 = Kristjánsson | first6 = G. | title = Gluten sensitivity in patients with IgA nephropathy. | journal = Nephrol Dial Transplant | volume = 24 | issue = 8 | pages = 2476-81 | month = Aug | year = 2009 | doi = 10.1093/ndt/gfp133 | PMID = 19332868 }}</ref>
*Esophageal [[squamous cell carcinoma]] - increased risk.<ref name=pmid11355914>{{Cite journal  | last1 = Messmann | first1 = H. | title = Squamous cell cancer of the oesophagus. | journal = Best Pract Res Clin Gastroenterol | volume = 15 | issue = 2 | pages = 249-65 | month = Apr | year = 2001 | doi = 10.1053/bega.2000.0172 | PMID = 11355914 }}</ref>
*Small bowel adenocarcinoma - increased risk.<ref name=pmid1060711>{{Cite journal  | last1 = West | first1 = RA. | last2 = McNeill | first2 = RW. | title = Maxillary alveolar hyperplasia, diagnosis and treatment planning. | journal = J Maxillofac Surg | volume = 3 | issue = 4 | pages = 239-50 | month = Dec | year = 1975 | doi =  | PMID = 1060711 }}</ref><ref name=pmid12940435>{{Cite journal  | last1 = Rampertab | first1 = SD. | last2 = Fleischauer | first2 = A. | last3 = Neugut | first3 = AI. | last4 = Green | first4 = PH. | title = Risk of duodenal adenoma in celiac disease. | journal = Scand J Gastroenterol | volume = 38 | issue = 8 | pages = 831-3 | month = Aug | year = 2003 | doi =  | PMID = 12940435 }}</ref>
*Thought to be related to the very rare [[collagenous sprue]] - controversial.<ref name=pmid21631278>{{Cite journal  | last1 = Zhao | first1 = X. | last2 = Johnson | first2 = RL. | title = Collagenous sprue: a rare, severe small-bowel malabsorptive disorder. | journal = Arch Pathol Lab Med | volume = 135 | issue = 6 | pages = 803-9 | month = Jun | year = 2011 | doi = 10.1043/2010-0028-RS.1 | PMID = 21631278 }}</ref><ref name=pmid23735026>{{Cite journal  | last1 = Busto-Bea | first1 = V. | last2 = Crespo-Pérez | first2 = L. | last3 = García-Miralles | first3 = N. | last4 = Ruiz-Del-Árbol-Olmos | first4 = L. | last5 = Cano-Ruiz | first5 = A. | title = Collagenous sprue: Don´t forget connective tissue in chronic diarrhea evaluation. | journal = Rev Esp Enferm Dig | volume = 105 | issue = 3 | pages = 171-174 | month = May | year = 2013 | doi =  | PMID = 23735026 }}</ref>
*[[Lymphocytic gastritis]] - seen in ~10% of individuals with celiac disease.<ref name=pmid9659261>{{Cite journal  | last1 = Feeley | first1 = KM. | last2 = Heneghan | first2 = MA. | last3 = Stevens | first3 = FM. | last4 = McCarthy | first4 = CF. | title = Lymphocytic gastritis and coeliac disease: evidence of a positive association. | journal = J Clin Pathol | volume = 51 | issue = 3 | pages = 207-10 | month = Mar | year = 1998 | doi =  | PMID = 9659261 }}</ref>


==Variants of celiac sprue==
==Variants of celiac sprue==
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===Serologic testing===
===Serologic testing===
*Anti-transglutaminase antibody.
*Anti-tissue transglutaminase (TTG) antibody -- >10 U/mL considered positive.<ref>URL: [http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/82587 http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/82587]. Accessed on: 13 August 2012.</ref>
**Alternative test: anti-endomysial antibody.
**Alternative test: anti-endomysial antibody.
*IgA -- assoc. with celiac sprue.
*Anti-gliadin antibodies.<ref name=pmid19629760>{{Cite journal  | last1 = Matthias | first1 = T. | last2 = Pfeiffer | first2 = S. | last3 = Selmi | first3 = C. | last4 = Eric Gershwin | first4 = M. | title = Diagnostic challenges in celiac disease and the role of the tissue transglutaminase-neo-epitope. | journal = Clin Rev Allergy Immunol | volume = 38 | issue = 2-3 | pages = 298-301 | month = Apr | year = 2010 | doi = 10.1007/s12016-009-8160-z | PMID = 19629760 }}</ref>
*IgA deficiency - associated with celiac sprue.


==Microscopic==
==Microscopic==
Features:<ref name=Ref_PBoD843>{{Ref PBoD|843}}</ref>
Features:<ref name=Ref_PBoD843>{{Ref PBoD|843}}</ref>
*Intraepithelial lymphocytes (IELs) - '''key feature'''.
*[[Intraepithelial lymphocytes]] (IELs) - '''key feature'''.
**Should be more pronounced at tips of villi.<ref name=pmid15280404>{{cite journal |author=Biagi F, Luinetti O, Campanella J, ''et al.'' |title=Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease? |journal=J. Clin. Pathol. |volume=57 |issue=8 |pages=835–9 |year=2004 |month=August |pmid=15280404 |pmc=1770380 |doi=10.1136/jcp.2003.013607 |url=}}</ref>
**Should be more pronounced at tips of villi.<ref name=pmid15280404>{{cite journal |author=Biagi F, Luinetti O, Campanella J, ''et al.'' |title=Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease? |journal=J. Clin. Pathol. |volume=57 |issue=8 |pages=835–9 |year=2004 |month=August |pmid=15280404 |pmc=1770380 |doi=10.1136/jcp.2003.013607 |url=}}</ref>
**Criteria for number varies:  
**Criteria for number varies:  
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*Mitosis increased (in the crypts).
*Mitosis increased (in the crypts).
*+/-Collagen band (pink material in mucosa) - "Collagenous sprue"; must encompass ~25% of mucosa.
*+/-Collagen band (pink material in mucosa) - "Collagenous sprue"; must encompass ~25% of mucosa.
Image:
*[http://commons.wikimedia.org/wiki/File:Coeliac_path.jpg Celiac sprue (WC)].


Notes:  
Notes:  
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*Flat lesions without IELs are unlikely to be celiac sprue.
*Flat lesions without IELs are unlikely to be celiac sprue.
*Mucosa erosions are rare in celiac sprue; should prompt consideration of an alternate diagnosis (infection, medications, Crohn's disease).
*Mucosa erosions are rare in celiac sprue; should prompt consideration of an alternate diagnosis (infection, medications, Crohn's disease).
*Biagi ''et al.''<ref name=pmid15280404>{{cite journal |author=Biagi F, Luinetti O, Campanella J, ''et al.'' |title=Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease? |journal=J. Clin. Pathol. |volume=57 |issue=8 |pages=835–9 |year=2004 |month=August |pmid=15280404 |pmc=1770380 |doi=10.1136/jcp.2003.013607 |url=}}</ref> count twenty cells in five (villi) tips.
===DDx===
*[[Giardiasis]].
**Have giardia organisms.
**Always consider ''Giardiasis'' and especially on exams.
*[[Crohn's disease]].
*[[Helicobacter gastritis]].<ref name=pmid16831028>{{Cite journal  | last1 = Brown | first1 = I. | last2 = Mino-Kenudson | first2 = M. | last3 = Deshpande | first3 = V. | last4 = Lauwers | first4 = GY. | title = Intraepithelial lymphocytosis in architecturally preserved proximal small intestinal mucosa: an increasing diagnostic problem with a wide differential diagnosis. | journal = Arch Pathol Lab Med | volume = 130 | issue = 7 | pages = 1020-5 | month = Jul | year = 2006 | doi = 10.1043/1543-2165(2006)130[1020:ILIAPP]2.0.CO;2 | PMID = 16831028 }}</ref>
*[[Cryptosporidiosis]].<ref name=pmid16831028/>
*[[Whipple's disease]] (very rare).
**Abundant macrophages should make one suspicious.
*[[Autoimmune enteropathy]] - pediatric population.
**Super duper rare.
*Drugs - e.g. olmesartan.<ref name=pmid24852741>{{Cite journal  | last1 = Fiorucci | first1 = G. | last2 = Puxeddu | first2 = E. | last3 = Colella | first3 = R. | last4 = Paolo Reboldi | first4 = G. | last5 = Villanacci | first5 = V. | last6 = Bassotti | first6 = G. | title = Severe spruelike enteropathy due to olmesartan. | journal = Rev Esp Enferm Dig | volume = 106 | issue = 2 | pages = 142-4 | month = Feb | year = 2014 | doi =  | PMID = 24852741 }}</ref>
*[[obesity|Morbid obesity]].<ref name=pmid17516730>{{Cite journal  | last1 = Harpaz | first1 = N. | last2 = Levi | first2 = GS. | last3 = Yurovitsky | first3 = A. | last4 = Kini | first4 = S. | title = Intraepithelial lymphocytosis in architecturally normal small intestinal mucosa: association with morbid obesity. | journal = Arch Pathol Lab Med | volume = 131 | issue = 3 | pages = 344; author reply 344 | month = Mar | year = 2007 | doi = 10.1043/1543-2165(2007)131[344b:IR]2.0.CO;2 | PMID = 17516730 }}</ref>
===Image===
<gallery>
Image: Celiac disease - low mag.jpg | CD - low mag. (WC/Nephron)
Image: Celiac disease - intermed mag.jpg | CD - intermed. mag. (WC/Nephron)
Image: Celiac disease - high mag.jpg | CD - high mag. (WC/Nephron)
Image: Celiac disease - very high mag.jpg | CD - very high mag. (WC/Nephron)
Image:Coeliac_path.jpg | Celiac sprue. (WC)
</gallery>


===Refractory sprue===
===Refractory sprue===
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| '''normal crypts'''
| '''normal crypts'''
|-
|-
| March 2
| Marsh 2
| hypertrophic crypts, IELs
| hypertrophic crypts, IELs
| '''normal villi'''
| '''normal villi'''
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*Villous atrophy can be assessed endoscopically.<ref name=pmid20844959>{{cite journal |author=Ciaccio EJ, Bhagat G, Tennyson CA, Lewis SK, Hernandez L, Green PH |title=Quantitative Assessment of Endoscopic Images for Degree of Villous Atrophy in Celiac Disease |journal=Dig Dis Sci |volume= |issue= |pages= |year=2010 |month=September |pmid=20844959 |doi=10.1007/s10620-010-1371-6 |url=}}</ref>
*Villous atrophy can be assessed endoscopically.<ref name=pmid20844959>{{cite journal |author=Ciaccio EJ, Bhagat G, Tennyson CA, Lewis SK, Hernandez L, Green PH |title=Quantitative Assessment of Endoscopic Images for Degree of Villous Atrophy in Celiac Disease |journal=Dig Dis Sci |volume= |issue= |pages= |year=2010 |month=September |pmid=20844959 |doi=10.1007/s10620-010-1371-6 |url=}}</ref>


==DDx==
==IHC==
*[[Giardiasis]].
*CD3 -- marks the IELs.<ref name=pmid15280404>{{cite journal |author=Biagi F, Luinetti O, Campanella J, ''et al.'' |title=Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease? |journal=J. Clin. Pathol. |volume=57 |issue=8 |pages=835–9 |year=2004 |month=August |pmid=15280404 |pmc=1770380 |doi=10.1136/jcp.2003.013607 |url=}}</ref>
**Have giarrdia organisms.
 
**Always consider ''Giardiasis'' and especially on exams.
==Sign out==
*[[Whipple's disease]] (very rare).
===TTG result not available===
**Abundant macrophages should make one suspicious.
<pre>
Duodenum, Biopsy:
- Small bowel mucosa with increased intraepithelial lymphocytes, villous
  architecture and crypt architecture within normal limits, see comment.
- Brunner's glands present.
- NEGATIVE for acute duodenitis.
- NEGATIVE for dysplasia.
 
Comment:
Focally, there are approximately 50 lymphocytes/100 enterocytes. Increased intraepithelial
lymphocytes is a nonspecific finding that may be seen in a number of conditions, including
infections (e.g. Helicobacter gastritis), obesity, and autoimmune disorders (e.g. Crohn's
disease).  It is seen in celiac disease; correlation with serology is suggested, if not done.
</pre>
 
====Block letters====
<pre>
SMALL BOWEL (DUODENUM), BIOPSY:
- SMALL BOWEL MUCOSA WITH INCREASED INTRAEPITHELIAL LYMPHOCYTES, VILLOUS
  ARCHITECTURE AND CRYPT ARCHITECTURE WITHIN NORMAL LIMITS, SEE COMMENT.
- NEGATIVE FOR ACUTE DUODENITIS.
- NEGATIVE FOR DYSPLASIA.
 
COMMENT:
There are approximately 45 lymphocytes/100 enterocytes. Increased intraepithelial
lymphocytes is a nonspecific finding. It is seen in celiac disease; correlation with TTG
serology is suggested, if not done.
</pre>
 
=====Alternate=====
<pre>
DUODENUM, BIOPSY:
- SMALL BOWEL MUCOSA WITH INCREASED INTRAEPITHELIAL LYMPHOCYTES, VILLOUS
  ARCHITECTURE AND CRYPT ARCHITECTURE WITHIN NORMAL LIMITS, SEE COMMENT.
- BRUNNER'S GLANDS PRESENT.
- NEGATIVE FOR ACUTE DUODENITIS.
- NEGATIVE FOR DYSPLASIA.
 
COMMENT:
Focally, there are approximately 50 lymphocytes/100 enterocytes. Increased intraepithelial
lymphocytes is a nonspecific finding that may be seen in a number of conditions, including
infections (e.g. Helicobacter gastritis), and autoimmune disorders (e.g. Crohn's disease). 
It is seen in celiac disease; correlation with TTG serology is suggested, if not done.
</pre>
 
===Positive TTG===
<pre>
DUODENUM, BIOPSY:
- SMALL BOWEL MUCOSA WITH BRUNNER'S GLANDS AND AN INCREASE OF INTRAEPITHELIAL LYMPHOCYTES
  (>50 LYMPHOCYTES/100 ENTEROCYTES), A PRESERVATION OF VILLOUS ARCHITECTURE AND CRYPTS
  WITHIN NORMAL LIMITS, SEE COMMENT.
 
COMMENT:
The findings are consistent with celiac disease, Marsh classification 1.
</pre>
 
<pre>
DUODENUM, BIOPSY:
- SMALL BOWEL MUCOSA WITH BRUNNER'S GLANDS, AN INCREASE OF INTRAEPITHELIAL LYMPHOCYTES
  (>60 LYMPHOCYTES/100 ENTEROCYTES), AND A BLUNTED VILLOUS ARCHITECTURE, SEE COMMENT.
 
COMMENT:
The findings are consistent with celiac disease, Marsh classification 3a.
</pre>
 
====Micro====
The sections show small bowel mucosa with Brunner's glands. Increased numbers of
intraepithelial lymphocytes are present ~ 50 lymphocytes/100 epithelial cells.
The villous architecture is within normal limits (no apparent villous blunting).
 
Neutrophils are present in the lamina propria; however, they are not found intraepithelial.
 
The epithelium matures normally to the surface (no dysplasia).


==See also==
==See also==
*[[Gastrointestinal pathology]].
*[[Gastrointestinal pathology]].
*[[Duodenum]].
*[[Duodenum]].
*[[Collagenous gastritis]].


==References==
==References==
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[[Category:Gastrointestinal pathology]]
[[Category:Gastrointestinal pathology]]
[[Category:Diagnosis]]
[[Category:Duodenum]]
Michael
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