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Unifying Concepts

Revised Criteria for the Diagnosis of Celiac Disease (CD) Proposed by the ESPGHAN

Definite diagnosis of CD

History and clinical presentation compatible with CD

  1. Serological screening compatible with CD: antigliadin antibody (AGA) antiendomysium antibody (AEA), tissue transglutaminase (tTG) antibody.
  2. Histological findings compatible with CD: villous atrophy
  3. Obvious clinical and serological response to a gluten free diet (GFD)
  4. Subject >2 years old
  5. Rule out other clinical conditions mimicking CD

Possible Clinical Manifestations of CD

A- Typical symptoms

  • Chronic diarrhea

  • Failure to thrive

  • Abdominal distention

B- Atypical symptoms

I- Secondary to malabsorption

  • Sideropenic anemia

  • Short stature

  • Osteopenia

  • Recurrent abortions

  • Hepatic steatosis

  • Recurrent abdominal pain

  • Gaseousness

II- Independent of malabsorption

  • Dermatitis herpetiformis

  • Dental enamel hypoplasia

  • Ataxia

  • Alopecia

  • Primary biliary cirrhosis

  • Isolated hypertransaminasemia

  • Recurrent aphthous stomatitis

  • Myasthenia gravis

  • Recurrent pericarditis

  • Psoriasis

  • Polyneuropathy

  • Epilepsy (with or without intracranial calcifications)

  • Vasculitis

  • Dilatative cardiomyopathy

  • Hypo/hyperthyroidism

Serologic Screening Tests for the Diagnosis of CD

Test

Sensitivity

Specificity

PPV

NPV

AGA IgG

57–100

42–98

20–95

41–88

AGA IgA

53–100

65–100

28–100

65–100

AEA IgA a

75–98

96–100

98–100

80–95

tTG IgA

90-98

95-97

a Patients older than 2 years.

Abbreviations: AEA, antiendomysium antibody; AGA, antigliadin antibody; CD, celiac disease; ESPGHAN, European Society of Pediatric Gastroenterology, Hepatology, and Nutrition; GFD, gluten-free diet; Ig, immunoglobulin; tTG, tissue transglutaminase; PPV, positive predictive value; NPV, negative predictive value.

 

 

References:

  1. Fasano A, Catassi C. Current approaches to diagnosis and treatment of celiac disease: an evolving spectrum. Gastroenterology. 2001 Feb;120(3):636-51. [Medline]
  2. Fasano A, Catassi C. Celiac disease.  N Engl J Med. 2012 Dec 13;367(25): 2419-26. [Medline]

Created: May 17, 2008

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