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Diagnosis is based on symptoms + blood tests + intestinal biopsy. A key challenge: gluten must be actively consumed at the time of testing — antibodies and biopsy findings normalise on a GFD.

Diagnosis often takes over a decade after symptom onset due to diverse presentations and low clinical awareness.

Pre-Test Requirements

  • Patient must be eating gluten (at least several weeks prior)
  • Those already on GFD must undergo a gluten challenge (>10g/day for 3 months, or until TG2 IgA becomes positive) before testing is reliable

Step 1: Blood Tests (Serology)

First-Line: TTG IgA

Tissue transglutaminase IgA (TTG IgA / TG2 IgA)

  • Sensitivity: 92.8% | Specificity: 97.9%
  • Cost-efficient, widely available
  • Severity of intestinal damage correlates with TTG IgA levels
  • Test simultaneously for IgA deficiency — more common in celiac (~1 in 40 patients vs ~1 in 500 general pop)

If IgA Deficient: IgG-Based Tests

  • DGP IgG (deamidated gliadin peptide)
  • EMA IgG (endomysial antibody)
  • TTG IgG — less accurate than IgA testing
  • IgA-based tests are unreliable in IgA deficiency

EMA (Endomysial Antibody)

  • Near 100% specificity; lower sensitivity
  • Costly, hard to interpret, observer-dependent
  • Useful to confirm borderline TTG IgA levels
  • Antigliadin antibodies (AGA)
  • Antireticulin antibodies (ARA) — superseded by more accurate tests

Paediatric Shortcut (ESPGHAN 2020)

Biopsy can be avoided in children if all three are present:

  1. Symptoms of celiac disease
  2. TTG IgA ≥10× upper normal limit
  3. Positive EMA antibody

Step 2: Endoscopy + Biopsy

Upper endoscopy with duodenal biopsy — beyond the duodenal bulb or from jejunum.

  • Multiple biopsies taken (patchy involvement → single biopsy may miss disease)
  • Endoscopic features: scalloping of folds, fissures, mosaic mucosa pattern, prominence of submucosal blood vessels, nodular mucosa
  • Capsule endoscopy — alternative for those unable/unwilling to undergo standard endoscopy

Marsh-Oberhuber Classification (Histology)

TypeIncreased IELs (>40/100 enterocytes)Crypt HyperplasiaVillous Atrophy
0 (normal)NoNoNo
1 (infiltrative)YesNoNo
2 (hyperplastic)YesYesNo
3a (destructive)YesYesMild
3b (destructive)YesYesModerate
3c (destructive)YesYesComplete

Note: These histological features are not exclusive to celiac — context (serology, clinical) is required.

Step 3: Genetic Testing (Optional)

HLA-DQ2 / HLA-DQ8 typing

  • Not needed for routine diagnosis
  • Useful to rule out celiac when borderline results (high negative predictive value — if neither DQ2 nor DQ8, celiac is very unlikely)
  • Helps decide whether to perform gluten challenge in those already on GFD

Differential Diagnosis

Conditions that can mimic celiac disease histologically or serologically:

Mimics with villous atrophy / elevated IELsPositive blood tests without villous atrophy
Tropical sprueErrors in blood collection
Eosinophilic gastroenteritisRecent infections
Lactose intoleranceCongestive heart failure
LymphomaChronic liver disease
Crohn's diseaseHypergammaglobulinaemia
H. pylori infection
Drug-induced (azathioprine, methotrexate, olmesartan, NSAIDs, PPIs)
Whipple disease, Giardiasis, tuberculosis
Zollinger–Ellison syndrome, HIV enteropathy
SIBO, collagenous sprue
CVID, autoimmune enteropathy

Non-Celiac Gluten Sensitivity (NCGS)

  • Intestinal + extraintestinal symptoms triggered by gluten
  • Faster onset and offset than celiac
  • Diagnosed by exclusion: rule out celiac and wheat allergy, then confirm resolution on GFD
  • No autoimmune markers; no villous atrophy

NICE Testing Indications

  • Persistent unexplained GI symptoms
  • Faltering growth
  • Chronic fatigue
  • Severe/persistent mouth ulcers
  • Unexplained iron, B12, or folate deficiency
  • Type 1 diabetes (at diagnosis)
  • Autoimmune thyroid disease (at diagnosis)
  • IBS in adults
  • First-degree relatives of celiac patients

Considered:

  • Metabolic bone disease
  • Unexplained neurological symptoms (neuropathy, ataxia)
  • Fertility problems / recurrent miscarriage
  • Persistently raised liver enzymes
  • Dental enamel defects
  • Down syndrome, Turner syndrome

Source Basis

Current synthesis incorporates guideline and guideline-summary sources including:

  • raw/american_college_of_gastroenterology_guidelines.17.pdf (American College of Gastroenterology Guidelines Update: Diagnosis and Management of Celiac Disease, 2023)
  • raw/practice-guidelines-celiac-disease.pdf (Diagnosis and Management of Celiac Disease: Guidelines From the American College of Gastroenterology, 2024)
  • raw/WJG-28-154.pdf (Current guidelines for the management of celiac disease, 2022)

These sources support the diagnostic sequence of serology while eating gluten, total IgA assessment, biopsy confirmation in most cases, selective pediatric non-biopsy pathways, and HLA testing mainly as an exclusion tool when results conflict or gluten has already been removed.

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