Category |
Manifestation |
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Life-threatening |
Toxic megacolon |
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complication |
Colonic perforation |
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Massive hemorrhage |
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Cancer-related |
Proven cancer |
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Presence of epithelial dysplasia in biopsy |
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Colonic stricture |
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Cancerophobia |
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“Unacceptable” |
Treatment refractoriness, |
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disease despite |
extra-colonic manifestation, |
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adequate |
chronic corticosteroid dependence |
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treatment |
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side effects/intolerance/complications from medication |
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(cataract, Cushing, osteoporosis, HTN, hyperglycemia) |
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Unacceptable life style |
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Whipple’s disease
• |
History |
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• |
Classical manifestations |
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- |
1907: Initial description |
- |
Polyarthritis |
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- |
Weight loss, diarrhea |
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- |
1961: Bacterial etiology |
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- |
Abdominal pain, |
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- |
2000: Culture and strain |
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malabsorption |
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isolation |
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Lymphadenopathy |
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- |
2002: Genome sequencing |
- |
Fever |
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- |
(2 strains) |
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• |
Neurological manifestations |
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2003: Culture in axenic |
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medium |
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- |
Memory disorder, dementia |
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Epidemiology |
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- |
Eye movement disorder |
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- |
Epilepsy |
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- |
<1,000 cases reported to |
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- |
date |
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• |
Cardiovascular |
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80 % of males |
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- |
manifestations |
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- |
40- to 50-old age group |
Endocarditis |
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- |
Predominantly Caucasian |
- |
Pericarditis |
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patients |
Trier, JS. Celiac Sprue and refractory sprue. In:-SleisengerMyocarditisand Fordtr n’ Gastro ntestinal and Liver Disease, 6th Ed, Feldman, M, |
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Scharscmidt, BF, Sleisenger, MH (Eds), Saunders, Philadelphia 1998. p. 1568 |
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-Rod-shaped morphology
-Size: 0.2 x 2 µm
-Trilamellar cell wall
Celiac Sprue
The celiac lesion in the proximal small intestine was first described by Paulley in 1954.
It was learned that celiac disease and adult non- tropical sprue share many of the same features
These classic findings are:
mucosal inflammation
crypt hyperplasia
villous atrophy
PAULLEY, JW. Observation on the aetiology of idiopathic steatorrhoea; jejunal and lymph-node biopsies. Br Med J 1954; 4900:1318
RUBIN, CE, BRANDBORG, LL, PHELPS, PC, TAYLOR, HC Jr. Studies of celiac disease. I. The apparent identical and specific nature of the duodenal and proximal jejunal lesion in celiac disease and idiopathic sprue. Gastroenterology 1960; 38:28
Many studies have also shown high prevalence
1:152 in the Belfast MONICA project evaluating 1,823 participants 1
1:256 was noted in a screening study of 1866 Swedish blood donors 2
1:99 in a study of 3654 Finnish students 3
1:96 in a study of 3188 Italian school children 4
1.Johnston, SD, Watson, RG, McMillan, SA, et al. Preliminary results from follow-up of a large-scale population survey of antibodies to gliadin, reticulin and endomysium. Acta Paediatr Suppl 1996; 412:61.
2.Grodzinsky, E. Screening for coeliac disease in apparently healthy blood donors. Acta Paediatr Suppl 1996; 412:36.
3.Maki, M, Mustalahti, K, Kokkonen, J, Kulmala, P. Prevalence of Celiac disease among children in Finland. N Engl J Med 2003; 348:2517.
4.Tommasini, A, Not, T, Kiren, V, et al. Mass screening for coeliac disease using antihuman transglutaminase antibody assay. Arch Dis Child 2004; 89:512
Celiac disease as an immune disorder that is triggered by an environmental agent (the gliadin component of gluten) in genetically predisposed individuals
Kagnoff, MF. Celiac disease. A gastrointestinal disease with environmental, genetic, and immunologic components. Gastroenterol Clin North Am 1992; 21:405. Schuppan, D. Current concepts of celiac disease pathogenesis. Gastroenterology 2000; 119:234.
Genetic factors play an important role- there is significantly increased risk of celiac among family members
A close association with the HLA-DQ2 and/or DQ8 gene locus has been recognized
HLA-DQ2 is found in 98% of celiac patients from Northern Europe.
However, ~25% of “normal” individuals in this population will also demonstrate HLA-DQ2
Kagnoff, MF. Celiac disease. A gastrointestinal disease with environmental, genetic, and immunologic components. Gastroenterol Clin North Am 1992; 21:4 Schuppan, D. Current concepts of celiac disease pathogenesis. Gastroenterology 2000; 119:234.
Petronzelli, F, Bonamico, M, Ferrante, P, et al. Genetic contribution of the HLA region to the familial clustering of coeliac disease. Ann Hum Genet 1997; 61:307 Houlston, RS, Ford, D. Genetics of coeliac disease. QJM 1996; 89:737.
Houlston, RS, Tomlinson, IP, Ford, D, et al. Linkage analysis of candidate regions for coeliac disease genes. Hum Mol Genet 1997; 6:1335