Celiac & Tropical Sprue



Celiac & Tropical Sprue





(Gastroenterology 2006;131:1977-80 & 2001;120:1526-40)


CELIAC SPRUE


Definition:



  • Intestinal reaction to alpha-gliadin in gluten » loss of villi & absorptive area


  • Gluten sensitive enteropathy, multisystem disease


Epidemiology:



  • 1% of population or 1 in 200 persons; Under diagnosed in U.S.


  • All Races, All Ages, All Genders


  • Inherited (70% of twins, 10% of relatives); First degree relative is a risk factor


Etiologies:



  • Antibodies directed against connective tissue or surface component of smooth muscle fibers


  • Associated disorders (many autoimmune):



    • Endocrine (DM, Thyroid, Addison’s, Osteopenia, Amenorrhea, Infertility)


    • Mixed connective tissue disease (Sjogren’s, RA)


    • Pulmonary (Asthma, Sarcoid)


    • Neurological (Seizures, Dementia, Peripheral neuropathy)


    • Skin (Dermatitis, Atopy, Psoriasis)


    • Malignancy (Lymphoma, Esophageal, Oropharyngeal)


    • Others: Down syndrome, Psychiatric disorders, Liver disease, IgA deficiency, IBD


Pathophysiology:



  • Genetics: HLA-DQ2 (95% of patients) and HLA-DQ8; Absence of the DQ gene rules out celiac disease with 99% confidence



    • However, DQ2 and 8 are present in 20-30% of the general Western population, suggesting other factors play a role


  • Environment: can affect any age of patients and since 70% (not 100%) of twins get the disease both suggest an environmental component


  • Histological stages: Type 0 (pre-infiltrative), Type 1 (infiltrative), Type 2 (hyperplastic), Type 3 (destructive), Type 4 (hypoplastic)



    • Type 3 usually begins symptoms; Type 4 is irreversible and is found in those not responding to gluten-free diet and lymphoma


Clinical Manifestations/Physical Exam:

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Aug 24, 2016 | Posted by in GASTROENTEROLOGY | Comments Off on Celiac & Tropical Sprue

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