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(re-arr, image, Marsh) |
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==Getting started== | ==Getting started== | ||
===PGY-2 DDx=== | |||
*Celiac. | *Celiac. | ||
**Intraepithelial lymphocytes - '''key feature'''. | **Intraepithelial lymphocytes - '''key feature'''. | ||
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**Too much blue and epithelium in the wrong place. | **Too much blue and epithelium in the wrong place. | ||
===Duodenal nodules=== | ===Duodenal nodules DDX=== | ||
{{familytree/start}} | {{familytree/start}} | ||
{{familytree | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | |A01=Duodenal<br>nodule}} | {{familytree | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | | | | | |A01=Duodenal<br>nodule}} | ||
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{{familytree/end}} | {{familytree/end}} | ||
=== | ===Normal duodenum=== | ||
*Three tall villi. | *Three tall villi. | ||
*Few intraepithelial lymphocytes; < 1 lymphocyte / 4 epithelial cells. | *Few intraepithelial lymphocytes; < 1 lymphocyte / 4 epithelial cells. | ||
*No subepithelial collagen band. | *No (pink) subepithelial collagen band. | ||
*Predominant lamina propria cell: plasma cells. | *Predominant lamina propria cell: plasma cells. | ||
**Lack of plasma cells suggests ''common variable immunodeficiency'' (CVID).<ref name=pmid20629103>{{cite journal |author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L |title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease |journal=Inflamm Bowel Dis |volume= |issue= |pages= |year=2010 |month=July |pmid=20629103 |doi=10.1002/ibd.21376 |url=}}</ref> | **Lack of plasma cells suggests ''common variable immunodeficiency'' (CVID).<ref name=pmid20629103>{{cite journal |author=Agarwal S, Smereka P, Harpaz N, Cunningham-Rundles C, Mayer L |title=Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease |journal=Inflamm Bowel Dis |volume= |issue= |pages= |year=2010 |month=July |pmid=20629103 |doi=10.1002/ibd.21376 |url=}}</ref> | ||
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==Celiac sprue== | ==Celiac sprue== | ||
===Etiology=== | ===General=== | ||
====Etiology==== | |||
*Autoimmune. | *Autoimmune. | ||
=== | ====Epidemiology==== | ||
== | |||
*Associated with: | *Associated with: | ||
**The skin condition ''[[dermatitis herpetiformis]]''.<ref>TN 2007 D22</ref> | **The skin condition ''[[dermatitis herpetiformis]]''.<ref>TN 2007 D22</ref> | ||
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**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). | ||
=== | ====Treatment==== | ||
*Gluten free diet. | |||
**''Mnemonic'': BROW = barley, rye, oats, wheat. | |||
====Serologic testing==== | |||
*Anti-transglutaminase antibody. | |||
**Alternative test: anti-endomysial antibody. | |||
*IgA -- assoc. with celiac sprue. | |||
===Microscopic=== | |||
Features:<ref name=Ref_PBoD843>{{Ref PBoD|843}}</ref> | Features:<ref name=Ref_PBoD843>{{Ref PBoD|843}}</ref> | ||
*Enteritis. | *Enteritis. | ||
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**Plasma cells. | **Plasma cells. | ||
**Macrophages. | **Macrophages. | ||
*Loss of villi - ''' | *Loss of villi - '''important feature'''. | ||
**Normal duodenal biopsy should have 3 good villi. | **Normal duodenal biopsy should have 3 good villi. | ||
*Mitosis increased (in the crypts). | *Mitosis increased (in the crypts). | ||
Image: | |||
*[http://commons.wikimedia.org/wiki/File:Coeliac_path.jpg Celiac sprue (WC)]. | |||
Notes: | Notes: | ||
*If you see acute inflammatory cells consider Giardiasis. | *If you see acute inflammatory cells, i.e. neutrophils, consider Giardiasis and other infectious etiologies. | ||
*Biopsy should consist of 2-3 sites. In children it is important to sample the duodenal cap, as it is the only affected site in ~10% of cases. | |||
DDx: | |||
*Giardiasis. | *Giardiasis. | ||
**Have giarrdia organisms. | **Have giarrdia organisms. | ||
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*Whipple's disease (very rare). | *Whipple's disease (very rare). | ||
**Abundant macrophages should make one suspicious. | **Abundant macrophages should make one suspicious. | ||
===Grading=== | |||
Most pathologists do not grade celiac sprue. | |||
The most common system is the ''modified Marsh system'':<ref name=pmid12145668>{{cite journal |author=Wahab PJ, Meijer JW, Dumitra D, Goerres MS, Mulder CJ |title=Coeliac disease: more than villous atrophy |journal=Rom J Gastroenterol |volume=11 |issue=2 |pages=121–7 |year=2002 |month=June |pmid=12145668 |doi= |url=}}</ref><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> | |||
{| class="wikitable" | |||
| | |||
| '''Marsh 1''' | |||
| '''Marsh 3A''' | |||
| '''Marsh 3C''' | |||
|- | |||
| Descriptors | |||
| Well-formed villi | |||
| Partial villous atrophy | |||
| Total villous atrophy | |||
|- | |||
| Alternate descriptors | |||
| Normal villous arch. | |||
| Blunted villi | |||
| Flattened mucosa | |||
|} | |||
==Giardiasis== | ==Giardiasis== |
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