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Difference between revisions of "Celiac sprue"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Celiac disease - high mag.jpg | ||
| Width = | | Width = | ||
| Caption = Celiac disease. [[H&E stain]]. | | Caption = Celiac disease. [[H&E stain]]. | ||
| Synonyms = celiac disease | |||
| Micro = Intraepithelial lymphocytes +/- villous blunting | | Micro = Intraepithelial lymphocytes +/- villous blunting | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[Giardiasis]], [[lymphoma]] ([[EATL]]) | | LMDDx = [[Giardiasis]], [[Enteropathy-associated T-cell lymphoma]] ([[EATL]]), [[inflammatory bowel disease]], [[MALT lymphoma]], others | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = | ||
| Line 15: | Line 16: | ||
| Grossing = | | Grossing = | ||
| Site = [[Duodenum]] | | Site = [[Duodenum]] | ||
| Signs = | | Assdx = [[dermatitis herpetiformis]], IgA deficiency, [[EATL]], [[duodenal adenocarcinoma]] | ||
| Syndromes = | |||
| Clinicalhx = improves with gluten free diet | |||
| Signs = diarrhea | |||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = uncommon | ||
| Bloodwork = TTG | | Bloodwork = TTG elevated (>10 U/mL) | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = [[normal duodenum]] | ||
| Tx = gluten free diet | |||
}} | }} | ||
'''Celiac sprue''', also '''celiac disease''', is a common pathology that affects the [[duodenum]]. | '''Celiac sprue''', also '''celiac disease''' (abbreviated '''CD'''), is a common pathology that affects the [[duodenum]]. | ||
It should not be confused with ''tropical sprue''. | It should not be confused with ''tropical sprue''. | ||
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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. | ||
== | ==General== | ||
* | *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: | ||
*[[Dermatitis herpetiformis]] - skin condition.<ref name=Ref_TN2007_D22>{{Ref TN2007| D22}}</ref> | *[[Dermatitis herpetiformis]] - skin condition.<ref name=Ref_TN2007_D22>{{Ref TN2007| D22}}</ref> | ||
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*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> | *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> | *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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==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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**Have giardia organisms. | **Have giardia organisms. | ||
**Always consider ''Giardiasis'' and especially on exams. | **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). | *[[Whipple's disease]] (very rare). | ||
**Abundant macrophages should make one suspicious. | **Abundant macrophages should make one suspicious. | ||
*[[Autoimmune enteropathy]]. | *[[Autoimmune enteropathy]] - pediatric population. | ||
**Super duper rare. | **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=== | ===Image=== | ||
<gallery> | <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) | Image:Coeliac_path.jpg | Celiac sprue. (WC) | ||
</gallery> | </gallery> | ||
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==Sign out== | ==Sign out== | ||
===TTG result not available=== | |||
<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> | <pre> | ||
DUODENUM, BIOPSY: | DUODENUM, BIOPSY: | ||
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The findings are consistent with celiac disease, Marsh classification 3a. | The findings are consistent with celiac disease, Marsh classification 3a. | ||
</pre> | </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:Diagnosis]] | ||
[[Category:Duodenum]] | |||