celiac disease
Synopsis
intestinal villous atrophy
increase of intraepithelial lymphocytes
intestinal crypt hyperplasia
chorionic lymphoplasmocytic infiltration
Differential diagnosis
other causes of intestinal villous atrophy
enteropathy-type T-cell lymphoma
Associations
autoimmune diabetes
autoimmune hepatitis
systemic autoimmune disorders (16133974)
Predisposition
trisomy 21 (10997372)
HLA class II genes explain 40% of the heritable risk.
non-HLA genes accounting for most of the familial clustering
Marsh classification of intestinal villous atrophy
| Types | Type 0 | Type 1 | Type 2 | Type 3a | Type 3b | Type 3c |
| IEL | <40 | >40 | >40 | >40 | >40 | >40 |
| Crypts | Normal | Normal | Hypertrophic | Hypertrophic | Hypertrophic | Hypertrophic |
| Villi | Normal | Normal | Normal | Mild atrophy | Marked atrophy | Absent |
NB: IEL, IntraEpithelial Lymphocytes (per 100 epithelial cells)
See also
refractory sprue (refractory celiac disease)
enteropathy-associated T-cell lymphoma
References
Oberhuber G, Granditsch G, Vogelsang H. The histopathology of coeliac disease: time for a standardized report scheme for pathologists. Eur J Gastroenterol Hepatol. 1999 Oct;11(10):1185-94. Review.PMID: 10524652
Reviews
Green PH, Cellier C. Celiac disease. N Engl J Med. 2007 Oct 25;357(17):1731-43. PMID: 17960014
Sollid LM, Jabri B. Is celiac disease an autoimmune disorder? Curr Opin Immunol. 2005 Dec;17(6):595-600. PMID: 16214317
Treem WR. Emerging concepts in celiac disease. Curr Opin Pediatr. 2004 Oct;16(5):552-9. PMID: 15367850
Farrell RJ, Kelly CP. Celiac sprue. N Engl J Med. 2002 Jan 17;346(3):180-8. PMID: 11796853
Vogelsang H, Schwarzenhofer M, Oberhuber G. Changes in gastrointestinal permeability in celiac disease. Dig Dis. 1998 Nov-Dec;16(6):333-6. PMID: 10207217
McManus R, Kelleher D. Celiac disease—the villain unmasked? N Engl J Med. 2003 Jun 19;348(25):2573-4. PMID: 12815145
Farrell RJ, Kelly CP. Celiac sprue. N Engl J Med. 2002 Jan 17;346(3):180-8. PMID: 11796853