Celiac Disease:Diagnosis and Control of Dietary Gluten

Shannon M. KaneBGSU Distance Dietetic Intern2012

Celiac Disease (CD)Also referred to as:

◦Gluten-sensitive enteropathy◦Gluten enteropathy◦Celiac sprue

What Is Celiac Disease?Genetic autoimmune disorderMediated by ingestion of glutenReaction to gluten inflicts

damage on the proximal small intestinal mucosa

May cause systemic organ damage and tissue atrophy

Affects 1% of the general worldwide population

Associated IssuesDermatitis HerpetiformisExtraintestinal disordersMalabsorption:

◦Associated with acidic, foul-smelling, watery steatorrhea diarrhea

◦May also be present even with normal fecal output

AnemiaHyperproteinemia

What is Gluten?Gluten is the storage protein in:

◦Wheat Bulgur, durum flour, farina, graham flour, kamut,

semolina, spelt, bromated flour, enriched flour, gluten flour, graham flour, phosphated flour, plain flour, self-rising flour, white flour, einkorn, emmer, wheat bran, wheat starch, wheat germ, cracked wheat, hydrolyzed wheat protein

◦Rye◦Triticale

A cross between wheat and rye

◦Barley Malt, malt flavoring, and malt vinegar

MisdiagnosisMany cases of CD go undiagnosed

◦Misdiagnosed as IBS, chronic fatigue, or other idiopathic disorders

◦Associated conditions are treated instead of underlying condition (which is CD)

◦Leads to long-term complications Organ damage Infertility Cancer

DiagnosisPositive serum antibodies

◦Immunoglobulin A (IgA)◦Anti-tissue transglutaminase (TTG)◦IgA antiendomysial antibodies (EMA)◦Villous atrophy of intestinal lining

Confirmed through intestinal biopsy

Many times CD is diagnosed by screening of at-risk groups or by endoscopy performed for other reasons

Characterizations of CDVillous atrophyCrypt hyperplasiaLymphocyte infiltrates of the

epithelium and lamina propria (mucous membrane)◦This effect leads to chronic diarrhea

and/or malabsorption

Responsible GenesHLA-DQ2

◦>90% of CD patients express this gene

HLA-DQ8◦Most of the remaining CD patients

express this geneCD almost exclusively occurs in

patients with positive HLA-DQ2/DQ8

Strong genetic component

Pathophysiology of CDIngestion of gluten (gliadins) Gliadins introduced to digestive

tractCD4 T cells rapidly proliferate

◦Found isolated in mucosa of CD patients

An α2-gliadin peptide is transported and released intact in the mucosa◦This triggers immune response◦Perpetuates intestinal inflammation

Associated ConditionsPrevalence of CD is greater in those with:

◦Family history of CD◦Autoimmune thyroid disease◦Down syndrome◦ IgA neuropathy◦Microscopic colitis◦Type 1 diabetes mellitus◦Dermatomyositis◦ IgA deficiency◦ Inflammatory arthritis◦Sjogren syndrome (dry eyes, dry mouth)

Clinical ManifestationsSymptoms are not uniform and vary with

age◦ Abdominal pain & distention◦ Diarrhea◦ Loss of appetite◦ Paleness of skin◦ Failure to thrive◦ Flatulence◦ Muscle wasting◦ Pale, bulky, or sticky stools◦ Vomiting◦ Weight loss◦ Lactose intolerance

Less Common Clinical Signs/SymptomsAlopeciaAtaxiaConstipationEpilepsyGERDHypoplasia

of dental enamel

Iron deficiency anemia

Obesity

Peripheral neuropathy

Short statureArthralgias,

arthropathyBehavioral

changesDermatitis

herpetiformis

Fatigue

Hepatic steatosis

Infertility, miscarriage

MyelopathyOsteoporosis

, osteopeniaRecurrent

apthous stomatitis

CD ProgressionClassical CD

◦Diagnosed in children aged 6-18 months

◦Villous atrophy◦Intestinal malabsorption

Atypical CD◦Minor intestinal symptoms◦Small intestine mucosal

abnormalities◦Osteoporosis, anemia, infertility,

neuropathies

CD Progression, con’tLatent CD

◦HLA-DQ2 and/or HLA-DQ8 genetic markers present

◦Serology may be positive, but mucosa normal

◦May or may not present with associated symptoms

◦Late activation of symptoms Cause for late activation remains elusive

CD Progression, con’tSilent CD

◦Mucosal abnormalities in the small intestine

◦Usually positive CD serology◦Asymptomatic

“Iceberg” Model of CDActive CD

◦Symptomatic◦Malabsorption present

Silent CD◦Asymptomatic◦Mucosal lesions present◦Serology usually positive

Latent CD◦No mucosal lesions◦Possibly symptomatic◦Serology may be positive

Prescription TreatmentCorticosteroids

◦BudesonideImmunosuppressant drugs

◦Azathioprine◦Reserved for pts with refractory CD

IV cladribine (0.1 mg/kg/d)

Dietary TreatmentGluten-free diet (GFD) is

necessary and the most effective treatment

CD is a lifelong condition which requires complete dietary exclusion of gluten-containing products

Following a GFD can normalize mucosal villous atrophy and improve intestinal inflammation

Gluten-Free DietRestrictive diet - excludes all

glutenAllowed foods:

◦Unprocessed beans, nuts, seeds◦Fresh eggs◦Fresh meats, poultry, and fish

Not breaded, marinated, or batter-coated

◦Fruits and vegetables◦Most dairy products

Gluten-Free Diet, con’t:Grains/Starches as part of GFD

AmaranthArrowrootBuckwheatCassavaLentilsPotatoSagoSeedsYuccaCorn/cornmeal

FlaxGluten-free flours

(rice, soy, corn, potato, bean)

Hominy (corn)MilletQuinoaRiceSorghumSoyTapiocaTeff

Gluten-Free Diet, con’t:Foods to Avoid (Unless Gluten-Free)BeerBreadsCakes and

piesCandiesCerealsCookies and

crackersCroutonsFrench friesGravies

Imitation meat or seafood

MatzoPastasProcessed

lunch meatSalad

dressingSauces

(including soy sauce)

Seasoned rice mixes

Seasoned snack foods (potato, tortilla chips)

Self-basting poultry

Soups and soup bases

Vegetables in sauce

Gluten-Free Diet, con’tBeware of products containing

gluten:◦Some grains can be contaminated

with gluten Oats

◦Food additives (malt flavoring), modified food starches

◦Medications, dietary supplements◦Play Doh (contains wheat)

Beware of cross-contamination within and outside of the home

Challenges of CD MaintenanceLearning cure associated with diseaseHigh-cost of gluten-free foodsFamily and friends must be

knowledgeableHealthcare provider education on CDWillingness of patient to follow GFDTrace amounts of gluten can be found

in “Gluten-Free” foodsCertain OTC/Rx meds contain glutenRelapse or refractory CD (RCD)

Dietary SuggestionsUse of “Alternative Medicines”

◦Supplement: Fat-soluble vitamins A,D,E, and K Vitamin B12 Folic acid Calcium Iron

◦Digestive enzymes Aids in breakdown of undigested gluten

proteins

◦Probiotics

Dietary Suggestions, con’tThose unaccustomed to a high

fiber diet should slowly introduce gluten-free whole grains/products◦Acknowledge contraindications for

fiberAvoid overuse of MVI above DRI’s

PreventionGood nutrition from birth

◦i.e. breastfeeding infants for extended time

◦Science is unsure if breastfeeding delays CD onset or in fact provides protection against the disorder

OTC testing kits becoming available◦Early intervention can reduce long-

term complicationsImmunomodulatory vaccine

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