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CELIAC SPRUE: AN UPDATE Sami N. Arslanlar, MD DIGESTIVE HEALTH ASSOCIATES OF TEXAS November 14, 2009. CASE. 53 year old white male presents with fatigue Daily diarrhea for years, peripheral neuropathy, osteoporosis No family history Drinks beer daily 117 lbs 5 feet 10 inches

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celiac sprue an update sami n arslanlar md digestive health associates of texas november 14 2009

CELIAC SPRUE: AN UPDATESami N. Arslanlar, MDDIGESTIVE HEALTH ASSOCIATES OF TEXASNovember 14, 2009

slide2
CASE
  • 53 year old white male presents with fatigue
  • Daily diarrhea for years, peripheral neuropathy, osteoporosis
  • No family history
  • Drinks beer daily
  • 117 lbs 5 feet 10 inches
  • Cachectic, guaiac positive
  • Hgb 7.6 Hct 26.2 MCV 72 Plt 612 Ferritin <10
  • Alb 1.8 INR 1.6 TB 0.1 AST 119 ALT 118 Alk phos 227 Calcium 5.6 Phos 1
slide3
CASE
  • Colonoscopy – normal
  • EGD – normal
  • Small bowel biopsies
    • severe villous atrophy with increased intraepithelial lymphocytes and lamina propria plasma cells
  • Serologies –
    • Anti endomysial IgA antibody +
    • Anti gliadin IgA antibody +
    • Anti gliadin IgG antibody +
slide4
CASE
  • Educated on gluten free diet
  • Improved clinically
  • Seen in clinic 6 months later with ascites
  • EGD, enteroscopy twice
  • Diagnosed with refractory celiac sprue
  • Treated with Prednisone
objectives
OBJECTIVES
  • What is celiac disease?
  • Will I ever see a patient with celiac disease?
  • Why do I need to know about it?
  • How do I diagnose celiac disease?
  • If I ever diagnose celiac disease, what should I worry about?
  • What is the treatment?
definition
DEFINITION
  • Gluten-sensitive enteropathy
  • Small intestinal malabsorption of nutrients after the ingestion of wheat gluten or related proteins from rye and barley
  • Characteristic villous atrophy of small intestinal mucosa
  • Prompt clinical and histologic improvement after strict gluten-free diet
  • Clinical relapse when gluten reintroduced
history
HISTORY
  • W.K. Dicke, Dutch pediatrician in early 1930s
  • Observation of relationship between celiac disease and bread
  • During WW II in Netherlands, food, particularly cereals, was scarce
  • Children with CD improved
  • At end of war, Swedish Air Forces supplied bread
  • Children with CD relapsed
celiac disease pediatrics
CELIAC DISEASE: PEDIATRICS
  • Classic presentation
    • Present 1-2 yo after cereals introduced in diet
    • Steatorrhea, vomiting, crampy abdominal pain
    • Failure to thrive, muscle wasting, abdominal distention
celiac disease in us
CELIAC DISEASE IN US
  • Prevalence is 1/3000
  • EMA + in 1/250 blood donors
  • Green et al surveyed support groups nationwide
    • Median age of diagnosis 46
    • Diagnosis delayed mean of 12 months but up to 10 years
    • Initial diagnoses
      • IBS
      • Psychological disorder
      • Fibromyalgia
the characters
THE CHARACTERS
  • HLA-DQ2, HLA-DQ8
  • Cereals
  • Gliadin
  • Tissue transglutaminase
gliadin
GLIADIN
  • Digestion studies reveal persistent 33-mer, residues 56-89
  • Carries multiple copies of three epitopes that are immunogenic in CS
  • Similar sequences in hordeins (barley) and secalins (rye)
  • Residues 57-68 and 62-75 stimulated all small intestinal gluten sensitive T cell clones from 4 adult celiac patients with HLA-DQ2
direct toxic effects of gliadin
DIRECT TOXIC EFFECTS OF GLIADIN
  • Reduction in number of junctional complexes altering intestinal permeability
  • Upregulation of HLA-DR expression
  • Changes in cell shape and size
  • Reduction in growth and viability
  • Decreased synthesis of nucleic acids and proteins
  • Cell damage mediated by lysosomes with signs of apoptosis

Similar changes seen in duodenal biopsy of CS patients

transglutaminase
TRANSGLUTAMINASE
  • Liver enzyme that incorporates amines into proteins
  • Mediators of biological glue
  • Factor XIIIa
  • Tranglutaminase I
    • Crosslinks proteins that comprise the cell envelope
transglutaminase ii ttg in disease
TRANSGLUTAMINASE II (tTG) IN DISEASE
  • Inclusion body myositis
  • Alzheimer’s disease
  • Parkinson’s disease
  • Progressive supranuclear palsy
  • Huntingtons disease
serological tests
SEROLOGICAL TESTS
  • Antigliadin (AGA) IgA
  • Antiendomysial (EMA) IgA
  • Antitissue transglutaminase (tTG) IgA
  • Total serum IgA level
  • Genetic testing
    • HLA DQ2 or HLA DQ8
diagnosis
DIAGNOSIS
  • Up to 3% of celiac patients have selective IgA deficiency
    • IgG serologies of utility in these patients
endoscopy
ENDOSCOPY

Normal duodenum

Scalloped folds

Mosaic pattern

slide29

BIOPSY

Normal

Partial atrophy I

Partial atrophy II

Partial atrophy III

Subtotal atrophy

Total atrophy

all villous atrophy is not celiac disease
Cows milk protein intolerance

Gastroenteritis

Giardiasis

Eosinophilic gastroenteritis

Bowel ischemia

Severe malnutrition

Diffuse small intestinal lymphoma

Autoimmune enteropathy

Hypogammaglobulinemia

Peptic duodenitis (Zollinger-Ellison syndrome)

Soy protein intolerance

Crohns disease

Small intestinal bacterial overgrowth

Radiation or cytotoxic chemotherapy

Tropical sprue

Kwashiorkor

Immunodeficiency syndromes

GVHD

Alpha chain disease

Refractory sprue

Collagenous sprue

All villous atrophy is not celiac disease ….
diagnostic dilemma
DIAGNOSTIC DILEMMA
  • ARE SEROLOGIES ADEQUATE FOR DIAGNOSIS?
  • ARE BIOPSIES ESSENTIAL?
  • ARE SEROLOGIES AND BIOPSIES BOTH NEEDED?
  • HOW MUCH EVIDENCE DO YOU NEED TO CONVINCE YOURSELF AND YOUR PATIENT OF THE DIAGNOSIS AND ITS NECESSARY TREATMENT?
celiac disease is a systemic disease
CELIAC DISEASE IS A SYSTEMIC DISEASE
  • GENERAL
  • GROWTH DELAY
  • MALIGNANCIES
  • ANEMIA
  • CNS
  • ATAXIA
  • SEIZURES
  • DEPRESSION
  • GI
  • DIARRHEA
  • VOMITING
  • DISTENTION
  • ABDOMINAL PAIN
  • MALNUTRITION
  • WEIGHT LOSS
  • HEPATITIS
  • CHOLANGITIS
  • HEART
  • CARDITIS
  • SKIN
  • DERMATITIS HERPETIFORMIS
  • APHTHOUS STOMATITIS
  • HAIR LOSS
  • BONE
  • OSTEOPOROSIS
  • ARTHRITIS
  • DENTAL ANAMOLIES
  • REPRODUCTIVE
  • MISCARRIAGE
  • INFERTILITY
malignancy
MALIGNANCY
  • Non-Hodgkin lymphoma
    • Main cause of mortality
    • Holmes reported a 43x increased risk
  • GI carcinomas
    • Esophageal
    • Small intestinal
  • Risk reduced with GFD
    • Returns to that of general population after 5 y
  • Special consideration if patient with known CD presents with exacerbation of symptoms
  • Enteropathy associated T-cell lymphoma
autoimmune disorders
AUTOIMMUNE DISORDERS
  • Prevalence of CD in patients with AIH 2.7-8%
  • Prevalence of PBC in CD patients up to 3%
    • 6% of patients with CD affected by PBC
  • CD found in 15% of Sjogren’s syndrome
  • Increased incidence of autoimmune disease in relatives of CD patients
  • Serologic testing of CD patients found up to 25% positive for other autoantibodies
    • Anti-ssDNA 14%
    • Anti-dsDNA 23%
    • Anticardiolipin 14%
osteoporosis
OSTEOPOROSIS
  • Most common extraintestinal manifestation after dermatitis herpetiformis
  • Found in 34% of adults at diagnosis
  • GFD does positively affect BMD
  • Mechanism
    • Calcium malabsorption
    • Vitamin D deficiency
    • Secondary hyperparathyroidism
    • Reduce muscle mass
    • Intestinal inflammation
endocrine disorders
DIABETES MELLITUS

1-1.5% of diabetic children have CD

Classical symptoms, poor glycemic control, frequent hypoglycemia

Associated with HLA-DR1-DQ2 and DR4-DQ8

THYROID DISEASE

Subclinical hypothyroidism very common

Increased prevalence of autoimmune thyroid disease

ENDOCRINE DISORDERS
treatment
TREATMENT

GLUTEN FREE DIET

GLUTEN FREE DIET

GLUTEN FREE DIET

GLUTEN FREE DIET

treatment41
Avoid all foods containing wheat, rye, & barley

Avoid all foods containing oats, (at least initially)

Use only rice, corn, maize, buckwheat, potato, soybean, or tapioca flours, meals or starches

Look for foods that have the gluten free symbol

Read all labels and study the ingredients of processed foods

Avoid all beers, lagers, ales, & stouts

Beware of gluten in medications, food additives, emulsifiers, & stabilizers

Wine, liquers, most ciders, & other spirits, including whiskey & brandy, are allowed!

TREATMENT
future treatments
FUTURE TREATMENTS
  • Bacterial endopeptidase
  • tTG inhibitors
  • Modification of cereal
  • Immunomodulators
conclusion
CONCLUSION
  • More common than previously thought
  • Presentation can be atypical
  • Serologic tests and biopsy for diagnosis
  • Associated conditions are numerous and can be affected by treatment
  • Gluten-free diet is effective treatment
resources
RESOURCES
  • Lone Star Celiac Support Group
    • dfwceliac.org
  • Celiac Sprue Association
    • csaceliacs.org
  • Celiac Disease Foundation
    • celiac.org
  • National Institutes of Health
    • digestive.niddk.nih.gov/ddiseases/pubs/celiac