Celiac sprue
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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
- Associated with:
- The skin condition dermatitis herpetiformis.[1]
- Tx: dapsone.
- IgA deficiency - 10-15X more common in celiac disease vs. healthy controls.[2]
- Risk factor for gastrointestinal T cell lymphoma - known as: enteropathy-associated T cell lymphoma (EATL).
- The skin condition dermatitis herpetiformis.[1]
Variants of celiac sprue
- Latent celiac sprue.
- ONLY intraepithelial lymphocytes.
- NO villous arch. change.
- Refractory celiac sprue.
- Subclassified - see microscopic.
- 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 (IELs) - key feature.
- 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
Many pathologists do not grade celiac sprue.
Marsh
The Marsh system, also Marsh-Oberhuber:[5]
| 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:[6]
| 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.[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
- ↑ TN 2007 D22
- ↑ 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.
- ↑ 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.
- ↑ 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/.
- ↑ 5.0 5.1 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.
- ↑ 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.
- ↑ 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)
- Serra S, Jani PA (November 2006). "An approach to duodenal biopsies". J. Clin. Pathol. 59 (11): 1133–50. doi:10.1136/jcp.2005.031260. PMC 1860495. PMID 16679353. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1860495/?tool=pubmed.