1. A Case of Abdominal Pain
Susan Poe, MD
9/22/04
11/7/2018

2. Introduction Learning Objective
to better recognize and manage a surprisingly common disease.
No financial support has been provided.
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3. Case 53 yo Caucasian woman C/o moderately severe RLQ pain
Duration 3 wks gradually worsening, now slightly lessened
Constant, nausea, sensation of fullness, worsened with movement, poor appetite
Missed work 1st time ever
Chills but no fever
Chronic constipation, sl looser stools lately
6 lb weight loss
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4. Case …. Pelvic u/s 2.4 cm R ovarian cystic fluid collection
Referred to UW gyn clinic
Repeat U/S no cyst
“no gynecologic cause”
Similar but less severe pain in the past
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5. Case …. PMH: anxiety, benign breast lump, PSH: BTL MEDS: xanax, colace
ALLG: sulfa, PCN, erythromycin
SH: Heavy smoker, factory worker, spouse with Alzheimer’s, grown son, minimal ETOH
GYN: postmenopausal, G3P1Ab2
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6. PE Alert, oriented,trim, tanned, older appearance than stated age
132/64, 66, wt 152
Chest: clear to A & P
CV: RRR no mgr
Abd: nl BS’s, soft, RLQ tender to deep palpation, sl guarding. No masses, HSM
GYN: nl uterus, no ovarian masses
Bimanual confirms, rectal heme neg
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7. stop
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8. Labs: WBC: 5.4 Hct: 40 LFTs, lytes, Cr: nl Amylase, lipase nl U/A nl
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9. stop
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10. Imaging
CT abd: “mass like appearance” to 2nd portion of duodenum, borderline enlarged mesenteric nodes, no retroperitoneal adenopathy, otherwise nl
CT pelvis: nl
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11. Procedure Upper endoscopy: negative gross appearance
Colonoscopy: metaplastic polyp
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12. Biopsy of duodenum: flat mucosa increased intraepithelial lymphocytes
C/w celiac sprue
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13. Celiac Disease
DEFINITION: lifelong inflammatory disease of small intestine caused by dietary gluten in genetically predisposed person
PATHOLOGY: Villous Atrophy: reduced villous height/ crypt ratio (nl 3-5 to 1), increased intraepithelial lymphocytes, extensive cell surface damage
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15. History 250 AD Aretaeus of Cappadocia:
1888 Samuel Gee : assoc with diet
1953 Wilheim Dicke: disease caused by wheat proteins
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16. Etiology GLUTEN: alcohol soluble fraction of wheat, rye,barley, oats
Wheat: gliadin
Rye: Secalins
Barley: Hordeins
Oats (less toxic): avenins
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17. Pathology Abnormal immune response of small intestine to gluten
Gluten absorbed into lamina propria
Sensitized T cells recognize gluten antigen in conjunction HLA DQ2 or DQ8 antigens
Activates other T cells, CD4
Release cytokines, other inflammatory products
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20. Epidemiology Previously thought to be disease of childhood
Adults increasingly recognized
Whites of Northern European descent
Age 10-40
1 per (previously thought 1 in 6000)
HLA association: DQA1.0501, DQB1.0201
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21. Prevalence 13,145 patients US
Tested endomysial Ab, positives biopsy confirmed
4508 1st deg relative: 1 in 22
3236 symptoms: 1 in 56
4126 no symptoms: 1 in 135
Arch Int Medicine 2003
Similar ratios in Sweden, Finland, Belfast
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22. Clinical
Classic (children): FTT, malabsorption, short stature, osteomalacia, rickets
Adults- usually more atypical
Often long standing before dx
Diarrhea usually
Wt loss
Abd pain: typically not severe, borborygmi, visible peristalsis, increased flatulence, bloating, large bulky pale stools
Lassitude
Symptoms of anemia
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23. Clinical… Short stature Osteoporosis, vitamin D deficiency
Deficiency of iron, ferritin, folic acid, B12, Ca, Mg, albumin
Amenorrhea, infertility
Spontaneous abortions
Lactose, sucrose intolerant
Bacterial overgrowth
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24. Disease Associations:
Increased mortality 4-fold
Increased risk GI malignancies
5-fold increased risk
T cell lymphoma: 10% associated with celiac dz , 10% survival
Metabolic bone disease
IgA deficiency 10%
Hyposplenism common
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25. Associations … Dermatitis Herpetiformis Aphthous ulcers DM 6-8%
24% of celiac patients
86% have celiac disease by bx
Itchy, burning vesicular rash, elbows, knees, buttocks,sacrum, face, neck, trunk or generalized
Dx: granular IgA deposits in nl skin
Aphthous ulcers
DM 6-8%
Hypothyroid, other autoimmune disease
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27. Diagnosis Serology IgA Antiendomysial Ab, IgA Transglutaminase ELISA
Sens %, spec 98-99%
IgA Transglutaminase ELISA
Sens 95%, spec 94%
IgA Antigliadin Ab less sens, spec
IgG Antigliadin Ab
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28. Diagnosis… Biopsy: Mandatory: 3 samples from duodenum
Marsh 1:intraepithelial lymphocytes
Marsh 2: mild villous atrophy
Marsh 3: completely flattened villi
Marsh 4: Severe villous atrophy, loss of crypts
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30. Diagnostic Criteria Abnormal Biopsy on gluten diet
Normalized on gluten free diet
Repeat Biopsy 3-4 months
Severity of lesion doesn’t correlate with severity of symptoms
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31. Treatment… Assess for associated conditions
CBC, Lytes, glucose, LFTs, Fe, Ferritin, B12, Vitamin D, TSH
DEXA scan
Pneumovax (debatable)
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32. Treatment… Gluten free diet lifelong Support group Internet sites
No wheat, barley, rye
Limit oats or not at all
Dietary counselling
Support group
Internet sites
Prevent bone loss
Correct nutritional deficiencies
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33. Treatment … Treat constipation (common) Monitor for response
Rice bran, ispaghula
Monitor for response
70% dramatic response within 2 wks
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34. Failure to respond 30% Continued gluten intake
Beer, malted breakfast cereals, snacks, sauces
Refractory disease (steroids)
Ulcerative jejunitis 33% mortality
Lymphoma
Chronic pancreatitis, pancreatic lesion
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35. Irritable Bowel 15% of general population Mayo Clinic Proc, April 2004
152 pts ,32 dyspepsia, 50 IBS, 15 both, 78 no sxms
Tested antibody levels, all negative
No association
Lancet, 2001
300 pts Rome II Criteria IBS
300 controls
4.7% (14) biopsy proven CE in IBS vs 0.7% (2) in controls
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36. Conclusion Common: 1 in 250-300
Association with irritable bowel Autoimmune response to gluten
Wide range of symptoms
Serology high sens and spec
Biopsy ,mandatory
Increased mortality 4-fold
Associated with lymphoma, nutritional deficiencies, osteoporosis, autoimmune disease,multiple other conditions
Easily treatable ?
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37. end
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