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