Celiac sprue
Celiac sprue 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]
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]
- Enteritis.
- Intraepithelial lymphocytes - key feature.
- Plasma cells.
- Macrophages.
- Loss of villi - important feature.
- Normal duodenal biopsy should have 3 good villi.
- 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.
Grading
Most pathologists do not grade celiac sprue.
The most common system is the modified Marsh system:[4][5]
| 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 |
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.
- ↑ Wahab PJ, Meijer JW, Dumitra D, Goerres MS, Mulder CJ (June 2002). "Coeliac disease: more than villous atrophy". Rom J Gastroenterol 11 (2): 121–7. PMID 12145668.
- ↑ 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.