An Update in the Diagnosis of Coeliac Disease

7/29/2019 An Update in the Diagnosis of Coeliac Disease

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AN UPDATE IN THE DIAGNOSISOF COELIAC DISEASE

7/29/2019 An Update in the Diagnosis of Coeliac Disease

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Coeliac disease

first time described in 1880

Link to the diet was established by Dickey in

1950 In 1954 Paulley found out that small intstinal

mucosa was abnormal.

Since this time biopsy is the routine practice.

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Definition

“an immune mediated enteropathy, whichimproves upon dietry exclusion of gluten”. 

Strong genetic predisposition HLA-DQ2 orHLADQ8 while the absence while theabsence of these molecules has a negativepredictive value for coeliac disease close to

100%.

Biopsy is the gold standard diagnostictechnique.

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Has the prevalence increased due

to better methods of diagnosis or

is this a real increase inpouplation?

Hygiene hypothesis

Swedish epidemic

Thus we are looking at a real increase in thedisease .

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Clinical feautures who to

test? Coeliac disease has a variable presentation.

recent genome-wide associaion studies have

shown that chronic inflammatory andautoimmune diseases are linked geneticallyto coeliac disease; e.g.type 1 DM,Gravesdisease and Crohn,s disease.

It is crucial that serological testing and biopsyare performed before any reduction in glutenintake.

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Iron deficiency anemia

British Society for gastroenterologyguidelines for investigation of IDArecommend that if serology has not beenperformed, a duodenal biopsy should betaken at endoscopy when this is performedto exclude a source of upper GI bleeding.

Even if other lesions which may explain IDAare found at endoscopy, a duodenal biopsyshould still be performed.

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Diagnosis –serology,endoscopy

and histopathology Serology first line.IgA(tTg) &IgA(EMA).

High level of sensitivity & specificity

PPV is 29% while sensitivity is 91%.

Serology will still miss the cases so biopsyshould be taken in any case.

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Endoscopy

Classic scalloping or subtle features of atrophy

Role of endomicroscopy for bettervisualization.

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Optimal site

Consensus opinion; 4 to 6 biopsies arenecessary for diagnosis.

originally second part of duodenum&beyond.

A three biopsy approach is sufficient; a fivebiopsy approach is best to detect the mostdegree of atrophy.

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Intraepithelial lymphocytes

IEL The finding of IEL in other parts of GIT should

alert one to the possibity of coeliac disease.

For example microscpoic colitis(lymphocyticcolitis &or/collagenous colitis) or lymphocyticgastritis/collagenous gastritis.

In RCD,diffuse lymphocytosis may occurthroughout GIT.

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BIOPSY ORIENTATION

Most UK and many US laboratories rely on agood orientation of biopsies.

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Which classification to use?

A major point to emphasize is that there is anunderstanding between clinician andhistopathologist as to which classification tobe used or whether a descriptive and possiblysemiquantitative report is preferred.

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ASSESSING ARCHITECTURE

Correct orientation of at least 3 to 4villi.

In the absence of villi, orientation can be

assessed by parallel crypts that reach frommuscularis mucosa to luminal surface.

Normal villous to cryp ratio is very variableand ranges from 3:1 to 5:1 but even 2:1,1.82:1

& 1:1 are sited as normal.

Atrophy is preceded by hyperplasia

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Intraepithelial lymphocytes

Increase is a cardinal diagnostic feature.

An even distribution along the entire lengthof the villi is even more common in coeliacdisease

The decrescendo sign with increased IEL inthe basal part of the villus with loss of IEL in

the tip & upper part of the villus is a pointer tosomething other than coeliac disease.

It is now agreed that 25 is normal but someeven suggest <20 as the norm.

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Use of IHC

Not a routine but may be useful in difficultcases where number is in doubt.

CD3+ in a top heavy pattern is suggetive of coeliac disease.

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Reporting

Description of villus architecture

IEL

Active inflammation ( neutrophils)

Pathogens if present

Comparison to a previous biopsy report

Conclusion: the features are suggestive of 

coeliac disease & serology should confirm thisdiagnosis whilst the patient is on glutencontaining diet.

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Mimics of coeliac histology

H pylori & bacterial overgrowth syndromemay show villous damage with a markedinflammatory infiltrate.

Treponema whipplei may be seen inmacrophages with PAS staining.

Rarely Giardia lamblia

Other mimics include protein deficiency(kwashiorkor) & tropical sprue.

This emphasizes rthe importance of clinicopathological correlation.

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Role of HLA typing

The patients in whom serology and biopsyare equivocal & the patient lacks the coeliacdisease at risk HLA haplotypes then coeliacdisease can be virtually excluded.

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FOLLOW UP

A normal tTg in coeliac patients on a glutenfree diet does not predict recovery.

Those patients with failure to heal seem to beprone to subsequent development of refractory coeliac disease and malignancies.

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Refractory coeliac disease

“Symptomatic malabsorption & villus atrophywhich persists despite scrupulous adherenceto a gluten free diet”. 

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ALGORITHM FOR DIAGNOSIS OF

COELIAC DISEASE

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