Celiac sprue

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Celiac sprue, also celiac disease (abbreviated CD), is a common pathology that affects the duodenum.

Celiac sprue
Diagnosis in short

Celiac disease. H&E stain.
Synonyms celiac disease
LM Intraepithelial lymphocytes +/- villous blunting
LM DDx Giardiasis, Enteropathy-associated T-cell lymphoma (EATL), inflammatory bowel disease, MALT lymphoma, others
Site Duodenum
Associated Dx dermatitis herpetiformis, IgA deficiency, EATL, duodenal adenocarcinoma
Clinical history improves with gluten free diet
Signs diarrhea
Prevalence uncommon
Blood work TTG elevated (>10 U/mL)
Clin. DDx normal duodenum
Treatment gluten free diet

It should not be confused with tropical sprue.

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.[1]

Epidemiology

Associated with:

Variants of celiac sprue

  1. Latent celiac sprue.
    • ONLY intraepithelial lymphocytes.
    • NO villous arch. change.
  2. Refractory celiac sprue.
    • Subclassified - see microscopic.
  3. Collagenous sprue.
    • Abundant mucosal collagen - see microscopic.

Clinical

Treatment

  • Gluten free diet.
    • Mnemonic: BROW = barley, rye, oats, wheat.

Serologic testing

  • Anti-tissue transglutaminase (TTG) antibody -- >10 U/mL considered positive.[11]
    • Alternative test: anti-endomysial antibody.
  • Anti-gliadin antibodies.[12]
  • IgA deficiency - associated with celiac sprue.

Microscopic

Features:[13]

  • Intraepithelial lymphocytes (IELs) - key feature.
    • Should be more pronounced at tips of villi.[14]
    • Criteria for number varies:
      • > 40 IELs / 100 enterocytes (epithelial cells).[15]
      • > 25 IELs / 100 enterocytes (epithelial cells).[16]
  • Loss of villi - important feature.
    • Normal duodenal biopsy should have 3 good villi.
  • Plasma cells - abundant (weak feature).
  • Macrophages.
  • Mitosis increased (in the crypts).
  • +/-Collagen band (pink material in mucosa) - "Collagenous sprue"; must encompass ~25% of mucosa.

Notes:

  • 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.
  • 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).
  • Biagi et al.[14] count twenty cells in five (villi) tips.

DDx

Image

  • CD - low mag. (WC/Nephron)

  • CD - intermed. mag. (WC/Nephron)

  • CD - high mag. (WC/Nephron)

  • CD - very high mag. (WC/Nephron)

  • Celiac sprue. (WC)

Refractory sprue

  • Type I: CD3 ~= CD8.
  • Type II: CD3 20% + less than CD8.

Grading

Many pathologists do not grade celiac sprue.

Marsh

The Marsh system, also Marsh-Oberhuber:[15]

Marsh score Descriptors Villi Intraepithelial
lymphocytes (IELs) 		Crypts
Normal (Marsh 0) normal normal villi < 40 / 100 epithelial cells normal crypts
Marsh 1 IELs increased normal villi > 40 / 100 epithelial cells normal crypts
Marsh 2 hypertrophic crypts, IELs normal villi > 40 / 100 epithelial cells hypertrophic crypts
Marsh 3a partial villous atrophy / blunted villi (mild) mild atrophy > 40 / 100 epithelial cells hypertrophic crypts
Marsh 3b moderate-to-marked villous atrophy /
blunted villi (moderate-to-marked) 		marked atrophy > 40 / 100 epithelial cells hypertrophic crypts
Marsh 3c total villous atrophy, flattened mucosa absent; flat as a pancake > 40 / 100 epithelial cells hypertrophic crypts

Simplified Marsh/Corazza

A simplified Marsh system - based (only) on villous architecture:[16]

Grade Similar Marsh grade Descriptors Alternate descriptors
A Marsh 1 well-formed villi, IELs > 25/100 enterocytes normal villous architecture
B1 Marsh 3a partial villous atrophy; villous-crypt ratio < 3:1 blunted villi
B2 Marsh 3c total villous atrophy flattened mucosa

Notes:

  • Villous atrophy can be assessed endoscopically.[20]

IHC

  • CD3 -- marks the IELs.[14]

Sign out

TTG result not available

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.

Block letters

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.
Alternate
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.

Positive TTG

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.

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.

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

References

  1. ↑ Singh, I.; Agnihotri, A.; Sharma, A.; Verma, AK.; Das, P.; Thakur, B.; Sreenivas, V.; Gupta, SD. et al. (Feb 2016). "Patients with celiac disease may have normal weight or may even be overweight.". Indian J Gastroenterol. doi:10.1007/s12664-016-0620-9. PMID 26892766.
  2. ↑ Greenwald, J.; Heng, M. (2007). Toronto Notes for Medical Students 2007 (2007 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. D22. ISBN 978-0968592878.
  3. ↑ Kumar, V.; Jarzabek-Chorzelska, M.; Sulej, J.; Karnewska, K.; Farrell, T.; Jablonska, S. (Nov 2002). "Celiac disease and immunoglobulin a deficiency: how effective are the serological methods of diagnosis?". Clin Diagn Lab Immunol 9 (6): 1295-300. PMID 12414763.
  4. ↑ Smerud, HK.; Fellström, B.; Hällgren, R.; Osagie, S.; Venge, P.; Kristjánsson, G. (Aug 2009). "Gluten sensitivity in patients with IgA nephropathy.". Nephrol Dial Transplant 24 (8): 2476-81. doi:10.1093/ndt/gfp133. PMID 19332868.
  5. ↑ Messmann, H. (Apr 2001). "Squamous cell cancer of the oesophagus.". Best Pract Res Clin Gastroenterol 15 (2): 249-65. doi:10.1053/bega.2000.0172. PMID 11355914.
  6. ↑ West, RA.; McNeill, RW. (Dec 1975). "Maxillary alveolar hyperplasia, diagnosis and treatment planning.". J Maxillofac Surg 3 (4): 239-50. PMID 1060711.
  7. ↑ Rampertab, SD.; Fleischauer, A.; Neugut, AI.; Green, PH. (Aug 2003). "Risk of duodenal adenoma in celiac disease.". Scand J Gastroenterol 38 (8): 831-3. PMID 12940435.
  8. ↑ Zhao, X.; Johnson, RL. (Jun 2011). "Collagenous sprue: a rare, severe small-bowel malabsorptive disorder.". Arch Pathol Lab Med 135 (6): 803-9. doi:10.1043/2010-0028-RS.1. PMID 21631278.
  9. ↑ Busto-Bea, V.; Crespo-Pérez, L.; García-Miralles, N.; Ruiz-Del-Árbol-Olmos, L.; Cano-Ruiz, A. (May 2013). "Collagenous sprue: Don´t forget connective tissue in chronic diarrhea evaluation.". Rev Esp Enferm Dig 105 (3): 171-174. PMID 23735026.
  10. ↑ Feeley, KM.; Heneghan, MA.; Stevens, FM.; McCarthy, CF. (Mar 1998). "Lymphocytic gastritis and coeliac disease: evidence of a positive association.". J Clin Pathol 51 (3): 207-10. PMID 9659261.
  11. ↑ URL: http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/82587. Accessed on: 13 August 2012.
  12. ↑ Matthias, T.; Pfeiffer, S.; Selmi, C.; Eric Gershwin, M. (Apr 2010). "Diagnostic challenges in celiac disease and the role of the tissue transglutaminase-neo-epitope.". Clin Rev Allergy Immunol 38 (2-3): 298-301. doi:10.1007/s12016-009-8160-z. PMID 19629760.
  13. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 843. ISBN 0-7216-0187-1.
  14. ↑ 14.0 14.1 14.2 Biagi F, Luinetti O, Campanella J, et al. (August 2004). "Intraepithelial lymphocytes in the villous tip: do they indicate potential coeliac disease?". J. Clin. Pathol. 57 (8): 835–9. doi:10.1136/jcp.2003.013607. PMC 1770380. PMID 15280404. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770380/.
  15. ↑ 15.0 15.1 Oberhuber G, Granditsch G, Vogelsang H (October 1999). "The histopathology of coeliac disease: time for a standardized report scheme for pathologists". Eur J Gastroenterol Hepatol 11 (10): 1185–94. PMID 10524652.
  16. ↑ 16.0 16.1 Corazza GR, Villanacci V, Zambelli C, et al. (July 2007). "Comparison of the interobserver reproducibility with different histologic criteria used in celiac disease". Clin. Gastroenterol. Hepatol. 5 (7): 838–43. doi:10.1016/j.cgh.2007.03.019. PMID 17544877.
  17. ↑ 17.0 17.1 Brown, I.; Mino-Kenudson, M.; Deshpande, V.; Lauwers, GY. (Jul 2006). "Intraepithelial lymphocytosis in architecturally preserved proximal small intestinal mucosa: an increasing diagnostic problem with a wide differential diagnosis.". Arch Pathol Lab Med 130 (7): 1020-5. doi:10.1043/1543-2165(2006)130[1020:ILIAPP]2.0.CO;2. PMID 16831028.
  18. ↑ Fiorucci, G.; Puxeddu, E.; Colella, R.; Paolo Reboldi, G.; Villanacci, V.; Bassotti, G. (Feb 2014). "Severe spruelike enteropathy due to olmesartan.". Rev Esp Enferm Dig 106 (2): 142-4. PMID 24852741.
  19. ↑ Harpaz, N.; Levi, GS.; Yurovitsky, A.; Kini, S. (Mar 2007). "Intraepithelial lymphocytosis in architecturally normal small intestinal mucosa: association with morbid obesity.". Arch Pathol Lab Med 131 (3): 344; author reply 344. doi:10.1043/1543-2165(2007)131[344b:IR]2.0.CO;2. PMID 17516730.
  20. ↑ Ciaccio EJ, Bhagat G, Tennyson CA, Lewis SK, Hernandez L, Green PH (September 2010). "Quantitative Assessment of Endoscopic Images for Degree of Villous Atrophy in Celiac Disease". Dig Dis Sci. doi:10.1007/s10620-010-1371-6. PMID 20844959.

External links

Review article(s)

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