Updated: 11/27/2018

Celiac Disease

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Snapshot
  • A 7-year-old girl is brought in by her parents for poor weight gain and recurrent diarrhea.  They have tried eliminating dairy from her diet with no improvement in symptoms.  She reports 3 - 5 loose stools per day for the last 6 months. The condition improves when she fasts and is usually worse when she eats fast food like pizza.  On exam, you notice several excoriated areas on the girls arms which she states are very itchy.
Introduction
  • A chronic hereditary intestinal malabsorption disorder caused by intolerance to gluten
    • a cereal protein found in wheat and rye and less so in barley and oats
  • Pathogenesis
    • gluten (specifically gliadin) becomes an antigen by acting as a substrate for tissue transglutaminase
      • tissue transglutaminase deaminates dietary gluten forming negatively-charged gluten fragments
    • fragments are phagocytosed by antigen presenting cells (APCs) and presented to T-helper cells
      • cytokines released promote aggregation of killer lymphocytes
    • lymphocytes cause mucosal damage with loss of villi and proliferation of crypt cells 
    • damage primarily occurs in the proximal small bowel
    • timing and dose of gluten when introduced into the diet has importance
  • Associated with
    • HLA-DQ2 and DQ8
    • chromosomal disorders
      • Down and Turner syndromes
    • other autoimmune diseases 
      • e.g., Hashimoto's thyroiditis and type I DM
  • Seen primarily in populations of northern European descent
Presentation
  • Symptoms
    • many may be asymptomatic
      • absent in children until they eat food containing gluten
    • diarrhea
    • failure to thrive
    • growth retardation
    • weight loss
    • weakness and pallor 
      • secondary to iron-deficiency anemia
  • Physical exam
    • classidermatitis herpetiformis  
      • pruritic, red, papulovesicular lesions on shoulders, elbows, knees 
      • inflammatory cutaneous disease with chronic relapsing course
Evaluation
  • Biopsy 
    • shows a pathognomonic blunting of intestinal villi and flat mucosa with hyperplastic glands and diffuse lymphocytic infiltration 
  • Serology
    • endomysial antibodies (EMA) 
      • show high sensitivity and specificity
      • proposed as screening test for celiac disease
    • tissue transglutaminase antibodies
      • also used for screening
    • antigliadin antibodies
Differential
  • Tropical sprue
    • infectious cause of sprue that can affect the entire small bowel
      • associated with travel to the tropics
    • responds to antibiotics
Treatment
  • Medical
    • lifestyle modifications and pharmacologic treatment
      • lifestyle modifications
        • avoid dietary gluten
        • dietary supplements
      • pharmacologic
        • corticosteroids in nonresponsive cases
        • treat dermatitis herpetiformis with dapsone 
Prognosis, Prevention, and Complications
  • Intestinal lymphoma
    • develops in 10-15% of patients
 

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Questions (8)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.GI.5) A 17-year-old male presents to his primary doctor complaining about an extremely itchy rash on his elbows and other extensor surfaces. This rash is shown in Figure A. Given the clinical associations of this rash, what other disease must be considered in this patient? Review Topic

QID: 103798
FIGURES:
1

Crohn disease

17%

(5/29)

2

Ulcerative colitis

10%

(3/29)

3

Celiac disease

69%

(20/29)

4

Juvenile rheumatoid arthritis

3%

(1/29)

5

Systemic lupus erythematosis

0%

(0/29)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(M1.GI.44) A 35-year-old Caucasian female presents with anemia, malaise, bloating, and diarrhea. Past genetic testing revealed that this patient carries the HLA-DQ2 allele. The physician suspects that the patient's presentation is dietary in cause. Which of the following findings would definitively confirm this diagnosis? Review Topic

QID: 101101
1

CT scan showing inflammation of the small bowel wall

5%

(1/20)

2

Biopsy of the duodenum showing atrophy and blunting of villi

85%

(17/20)

3

Biopsy of the colon showing epithelial cell apoptosis

0%

(0/20)

4

Liver biopsy showing apoptosis of hepatocytes

0%

(0/20)

5

Esophageal endoscopy showing lower esophageal metaplasia

5%

(1/20)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(M1.GI.77) A 30-year-old Caucasian female presents with weight loss, pallor, and periodic abdominal discomfort. Physical exam reveals pruritic, red lesions on the patient’s shoulders, elbows, and knees. An endoscopic duodenal biopsy is taken and shown in Figure A. Which of the following diseases is associated with the patient’s condition? Review Topic

QID: 101134
FIGURES:
1

Zollinger-Ellison syndrome

18%

(39/211)

2

Hirschsprung’s disease

5%

(10/211)

3

Peutz-Jehger’s polyposis

18%

(39/211)

4

Hashimoto’s thyroiditis

49%

(103/211)

5

Helicobacter pylori infection

9%

(18/211)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(M1.GI.26) A 22-year-old woman presents to her primary care physician complaining of a red, itchy rash on her elbows and shoulders for 2 months. She has no history of medical problems, and review of systems is positive only for occasional loose stools. She is appropriately prescribed dapsone, which relieves the rash within hours. What is the diagnosis? Review Topic

QID: 104237
1

Candida intertrigo

3%

(3/115)

2

Porphyria cutanea tarda

17%

(20/115)

3

Systemic lupus erythematousus

7%

(8/115)

4

Dermatitis herpetiformis

49%

(56/115)

5

Leprosy

20%

(23/115)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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