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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

57100

4298

2095

4188

AGA IgA

53100

65100

28100

65100

AEA IgA a

7598

96100

98100

8095

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]

Created: May  17, 2008
Last Modified: 05/17/2008