Celiac Disease in Primary Care

Dustin M Adkins

Spring 2007

Essentials of Celiac Disease Autoimmune disorder triggered by gluten “Gluten” is a collective term for the storage

proteins of wheat, rye, and barley. Adaptive/innate immune response damages

villi in the proximal small intestine Not just GI complaints! A multisystem

disorder with highly variable presentation Increased risk of Non Hodgkins Lymphoma

(2.7-6.3x) and overall mortality (1.9-3.4x)

Epidemiology

Not as “rare” as once thought

Affects 1:100 in USA (AGA 2007)

Under-diagnosed

Classical (Textbook) Celiac Disease Symptoms and complications of

malabsorption Hallmark: Diarrhea/steatorrhea

(chronic) Abdominal distension, edema,

extreme lethargy, weight loss, failure to thrive

Onset at any age, gradual or rapid Often fulfills criteria for IBS. Be

suspicious of refractory IBS-D especially with associated celiac symptoms!

http://www.pigur.co.il/imgceliac/celiac.jpg

http://webedit.caregroup.org/content/bidmc/Departments/Medicine/Gastroenterology/images/DHimage.jpghttp://pathmicro.med.sc.edu/ghaffar/mhcderm.jpghttp://merck.micromedex.com/images/bpm/BPM01DE05F09.gif

Dermatitis Herpetiformis is “classically” associated with Celiac

Disease. Only in a minority of patients.

Atypical Celiac Disease

MOST COMMON presentation Extra-intestinal manifestations dominate Blood, Bones, & Babies

Iron deficiency anemia: Unexplained or iron-therapy-refractory (2.3%-5.0%)

Osteoporosis: Premature onset (1.0%-3.4%) Infertility: Unexplained, recurrent fetal loss

(2.1%-4.1%) Many other S/Sx: short stature, fatigue,

delayed puberty, vitamin deficiencies…

Diarrhea43%

Screening17%

Incidental10%

Anemia8%

Bone Disease6%

Wt Loss6%

Abd Pain5%

Other5%

Clinical Presentation of Celiac Disease

Summary of the clinical presentation of celiac disease. Included 170 biopsy-diagnosed celiac disease patients diagnosed between 1993 and 2000 (Lo 2003).

Asymptomatic Celiac Disease Often detected

when screening 1° relatives, or incidentally during EGD

Risk of complications, lymphoma, mortality still exists!

Associated Disorders (HLA DQ2/DQ8) Autoimmune (10-fold ↑)

Thyroiditis (3%) Type I Diabetes (1-12%) Sjögren’s syndrome Addison’s disease Autoimmune liver

disease Cardiomyopathy

Other related disorders Down syndrome (3-12%) Turners syndrome Williams syndrome Ulcerative colitis Crohns disease IgA nephropathy Occipital calcifications Neuropsychiatric d/o’s

Diagnosis

Anti-tTG IgA is the single most effective test for PCPs! (95% Sens; 98% Spec)

Genetic tests can only rule out Celiac disease (HLA DQ2/DQ8)

Duodenal biopsy (EGD) remains the Gold Standard

http://www.glutenfreeworks.com/gluten_explained.phphttp://www.bidmc.harvard.edu/display.asp?node_id=7715

Treatment: Lifelong Gluten-Free Diet Difficult task for the

patient. Should see a registered

dietician Complicated by fast-

food lifestyles, hidden ingredients, poor labeling, and costly/unavailable specialty foods.

Lifelong diet normalizes mortality/comborbidity risks

Never advise a patient to start a Gluten-Free

Diet before biopsy!

It alters the histopathology

requiring additional testing ($$$),

and ticks off the gastroenterologist!

Lifelong care for the Celiac Patient Take a good history! Symptom improvement

doesn’t mean all gluten has been removed from the diet.

Make sure the patient has support Celiac support groups Tons of online info on gluten-free dieting

Work with pharmacist to avoid gluten in drugs Nardil, Humira, Flonase, Claritin…look it up! Inactive ingredients (dextri-maltose, dusting powder,

starches) Watch out for anemia, osteoporosis, infertility, and

vitamin deficiencies

Participation time! Which is gluten-free?

Modified

Modified

Food Starch

Food Starch

MaltMalt

Restaurant Restaurant Contamination?Contamination? Wheat

Wheat