Difference between revisions of "Celiac sprue"

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Most pathologists do not grade celiac sprue.
Most pathologists do not grade celiac sprue.


The most common system is the ''Marsh system'' (abbreviated):<ref name=pmid10524652>{{cite journal |author=Oberhuber G, Granditsch G, Vogelsang H |title=The histopathology of coeliac disease: time for a standardized report scheme for pathologists |journal=Eur J Gastroenterol Hepatol |volume=11 |issue=10 |pages=1185–94 |year=1999 |month=October |pmid=10524652 |doi= |url=}}</ref><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>
The (full) ''Marsh system'', also ''Marsh-Oberhuber'':<ref name=pmid10524652>{{cite journal |author=Oberhuber G, Granditsch G, Vogelsang H |title=The histopathology of coeliac disease: time for a standardized report scheme for pathologists |journal=Eur J Gastroenterol Hepatol |volume=11 |issue=10 |pages=1185–94 |year=1999 |month=October |pmid=10524652 |doi= |url=}}</ref><ref name=pmid17544877>{{cite journal |author=Corazza GR, Villanacci V, Zambelli C, ''et al.'' |title=Comparison of the interobserver reproducibility with different histologic criteria used in celiac disease |journal=Clin. Gastroenterol. Hepatol. |volume=5 |issue=7 |pages=838–43 |year=2007 |month=July |pmid=17544877 |doi=10.1016/j.cgh.2007.03.019 |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
|}
 
The ''Marsh system'' (full):<ref name=pmid10524652>{{cite journal |author=Oberhuber G, Granditsch G, Vogelsang H |title=The histopathology of coeliac disease: time for a standardized report scheme for pathologists |journal=Eur J Gastroenterol Hepatol |volume=11 |issue=10 |pages=1185–94 |year=1999 |month=October |pmid=10524652 |doi= |url=}}</ref>
{| class="wikitable"
{| class="wikitable"
| Marsh score
| Marsh score
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| '''hypertrophic crypts'''
| '''hypertrophic crypts'''
|-
|-
| Marsh 3A
| Marsh 3a
| partial villous atrophy / blunted villi (mild)
| partial villous atrophy / blunted villi (mild)
| '''mild atrophy'''
| '''mild atrophy'''
Line 109: Line 91:
| hypertrophic crypts
| hypertrophic crypts
|-
|-
| Marsh 3B
| Marsh 3b
| moderate-to-marked villous atrophy /<br> blunted villi (moderate-to-marked)
| moderate-to-marked villous atrophy /<br> blunted villi (moderate-to-marked)
| '''marked atrophy'''
| '''marked atrophy'''
Line 115: Line 97:
| hypertrophic crypts
| hypertrophic crypts
|-
|-
| Marsh 3C
| Marsh 3c
| total villous atrophy, flattened mucosa
| total villous atrophy, flattened mucosa
| '''absent'''; flat as a pancake
| '''absent'''; flat as a pancake
Line 121: Line 103:
| hypertrophic crypts
| hypertrophic crypts
|}
|}
A modified ''Marsh system'' - based only on villous architecture:<ref name=pmid17544877>{{cite journal |author=Corazza GR, Villanacci V, Zambelli C, ''et al.'' |title=Comparison of the interobserver reproducibility with different histologic criteria used in celiac disease |journal=Clin. Gastroenterol. Hepatol. |volume=5 |issue=7 |pages=838–43 |year=2007 |month=July |pmid=17544877 |doi=10.1016/j.cgh.2007.03.019 |url=}}</ref>
{| class="wikitable"
| Grade (Marsh grade)
| Descriptors
| Alternate descriptors
|-
| A (Marsh 1)
| well-formed villi, IELs > 25/100 enterocytes
| normal villous architecture
|-
| B1 (Marsh 3a)
| partial villous atrophy
| blunted villi
|-
| B3 (Marsh 3c)
| total villous atrophy
| flattened mucosa
|}


Notes:
Notes:

Revision as of 16:15, 7 February 2011

Celiac sprue, also celiac disease, is a common pathology that affects the duodenum.

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

Etiology

  • Autoimmune.

Epidemiology

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-transglutaminase antibody.
    • Alternative test: anti-endomysial antibody.
  • IgA -- assoc. with celiac sprue.

Microscopic

Features:[3]

  • Intraepithelial lymphocytes - key feature.
    • Should be more pronounced at tips of villi.[4]
  • 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.

Image:

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).

Refractory sprue

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

Grading

Most pathologists do not grade celiac sprue.

The (full) Marsh system, also Marsh-Oberhuber:[5][6]

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
March 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

A modified Marsh system - based only on villous architecture:[6]

Grade (Marsh grade) Descriptors Alternate descriptors
A (Marsh 1) well-formed villi, IELs > 25/100 enterocytes normal villous architecture
B1 (Marsh 3a) partial villous atrophy blunted villi
B3 (Marsh 3c) total villous atrophy flattened mucosa


Notes:

  • Villous atrophy can be assessed endoscopically.[7]

DDx

  • Giardiasis.
    • Have giarrdia organisms.
    • Always consider Giardiasis and especially on exams.
  • Whipple's disease (very rare).
    • Abundant macrophages should make one suspicious.

See also

References

  1. ↑ TN 2007 D22
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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/.
  5. ↑ 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.
  6. ↑ 6.0 6.1 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.
  7. ↑ 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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