Cœliaque disease

The coeliaque disease (sometimes called cœliaquie or intolerance with the gluten or sprue ), is an auto-immune disease, characterized by an attack of whole or part of the villosities recovering the Small intestine . This disease is due to an intolerance to the Gluten and related Protéine S which one finds in some Céréale S (Blé, Seigle, Orge, German wheat, oats). It results a Malabsorption from it and thus from the food Carence S. The oats east cannot be not in question: it would be simply about a contamination by the fields " muticulture" such that they exist in Europe. The cœliaques people must abstain from with life consuming the products containing these glutens, which allows a complete regression of the symptoms of the disease.

Causes

There exists a clear family predisposition to the cœliaque disease whose prevalence is about 10% in the parents of first degree of a patient reached. The disease is present primarily only at patients carrying certain groups HLA (HLA-DQ2 or HLA-DQ8) but the presence of these groups do not mean, for as much, being carrying the disease.

Apart from these genetic predispositions, other factors environmental, probably infectious, viral and/or bacterial still badly known, are likely to start the active phase of the disease (the responsibility for a Rotavirus is sometimes blamed).

Other factors could also have a role a such too early introduction of the gluten into the food mode of the infant. The maternal Allaitement would be, on the contrary, a protective factor.

The cœliaque disease has two peaks of frequency with a revelation either in early childhood, generally between six months and two years or after the introduction of the gluten into the food mode, or to the adulthood generally between 20 and 40 years. The forms with late revelation (after 65 years) are however not rare.

Physiopathology

The Gluten is a protein being in many cereals. It is transformed into gliadine in the digestive tract. The penetration of the latter in the intestinal wall at predisposed subjects would cause an inflammatory reaction involving the destruction of the cells composing intestinal villosities. This intolerance with the gluten is of origin immunological, but without reaction of Hypersensibilité. It is thus not a Allergie.

With the active phase of the disease, before the diagnosis and thus the introduction of a mode excluding the gluten, the majority of the patients have a total atrophy or subtotale villosities (disappearance of these last) on the level of the intestinal wall with a clear increase in the number of Lymphocyte S in the epithelium of the latter.

This atrophy is prevalent on the level of the Small intestine proximal but can, when the attack is important, visible being on the whole of the small intestine. She leads then to a more or less total syndrome of malabsorption with in particular a malabsorption of the Vitamine B12 on the level of hail distal. The blood rate of the latter then is very lowered.

Epidemiology

One generally finds this disease at the child, but the diagnosis perhaps made in the adult when the disease was discrete during childhood.

This disease touches especially the populations of Northern Europe. Estimates of its frequency among the people of European origin vary between 1 out of 300 and 1 out of 500. Among Irish, the prevalence would reach 1 out of 132, while the average prevalence in the United Kingdom would be of 1 out of 100. This different prevalence is partly due to the disparity of the diagnoses carried.

Because of its partly genetic nature, the doctors generally recommend that the close relatives of patients reached pass a test even if they do not present any symptom.

It is 2 to 3 times more frequent at the woman. This sex-ratio currently attenuates, with the increase in the rate of diagnosis of the clinically asymptomatic forms (without apparent sign).

Signs and symptoms

The most frequent signs are:

a fatigability (Asthenia),
of the abdominal pains and a diarrhea of the malabsorption type with Stéatorrhée (lubricating saddles), slimming and denutrition.

One can also find anomalies biological which are indirect signs of malabsorption on the level of hail:

Anemia associated with a deficiency out of iron, folates, B12 vitamin;
Deficit in some factors of coagulation (factors II, VII and X);
hypoprotidemy (drops blood protein concentration) with hypo-albuminemy;
deficit out of calcium (Hypocalcémie), out of magnesium, zinc;
reduction in the blood cholesterol concentration (hypocholesterolemy).

One does not find this table only at 20% of the patients. There can also be many very different symptoms.

The atypical forms or frustrate are frequent. They are more frequently observed in the adult. Their atypical character often involves a delay with the diagnosis. One can thus observe:

an anemia by iron deficit, even a simple iron deficit without anemia,
a repeating Aphtose oral,
of the menstrual disorders such as a Amenorrhoea primary or secondary or a sterility,
of the abortions with repetition,
a fetal hypotrophie,
an epilepsy or a Ataxia,
of the osseous or articular pains unexplained,
a Osteoporosis without found cause, associated or not with fractures,
an unexplained increase in the Transaminases in blood.

Diagnosis

The cœliaque diagnosis of disease is often easy when there are digestive clinical signs. In their absence, the diagnosis is much more complex.

It rests on three principal criteria: serologic, histological and effectiveness of the exclusion of the gluten.

There exists some characteristic in the adult.

On the one hand, it is essential not to ignore the disease in front of digestive or extra-digestive demonstrations which, untreated, could involve serious complications, such as a denutrition or a neurological attack. In addition, to pose the diagnosis at a patient will have for him very important consequences since that will imply the total and final suppression gluten in the food, therefore a very constraining mode. It is thus necessary all at the same time to easily evoke this diagnosis in front of symptoms which are not inevitably typical and evocative, but also to collect sufficient arguments to be certain diagnosis.

Serology

The proportioning of the Anticorps (IgA anti transglutaminase tissue, and, in a less reliable way, anti-gliadine IgA) in blood is of a great sensitivity and specificity. It is useful for the diagnosis and the follow-up of the mode without gluten. This proportioning is however less sensitive in the event of little advanced forms of the disease.

Biopsy duodénale

The Fibroscopie can show nonspecific anomalies: rarefied folds of the mucous membrane, aspect in mosaic… Even in the event of normality of the aspect, a confirmation must be made by biopsy of the mucous membrane duodénale.

It must be multiple (4 to 6 samples).

A nutritional assessment must be made comprising the proportioning of the blood concentration out of iron, calcium, Folic acid, Vitamine B12.

A Ostéoporose (demineralization of the bones with increased risks of fractures) must be systematically required.

Complications

Until a third of the cœlic diseases answer in an imperfect way the mode. Other drugs were tested with a variable success: Infliximab, auto- Greffe of marrow… the forecast of this proliferative form remains however dark with a survival lower than 5 years.

The cœliaque disease is a traditional risk factor of occurred of a Cancer of the small intestine (adenocarcinomist), rare disease. This data is however constestée.

Authentic Lymphome S can however develop on cœeliaques diseases not refractories. They are localized on the level of the digestive tract but can occur elsewhere. They occur after about sixty and must be suspectés during an aggravation of the signs on a disease, until stabilized there.

Treatment

The only treatment remains to follow a mode excluding any source from gluten in the food.

The mode without gluten must be recommended with life (there is no regression of the disease), in particular in the adult, because it partly prevents the risk of occurred of Lymphome (blood cancer) and of osseous complications.

The gluten is primarily in the flours. The patient can use products stamps “without gluten”. He is not restricted on the level fruit and vegetables like for the meat, the fish, the dairy produces.

It is necessary to control the effectiveness of the mode and the good follow-up by the observation of the clinical and biological improvement after one to three months of mode, by the regression of the anomalies to the biopsy (atrophies villositaire) and the negativation of the specific antibodies after one year of mode.

It is also necessary to supervise the disappearance of the symptoms which had directed the diagnosis (osseous problems,…). Complements vitamin can be prescribed when the health condition of the patient asks it.

Treatments, containing absorbtion of enzymes making it possible to degrade the gluten, and thus, theoretically to avoid the mode, are under development.

Exceptionally, the gluten reintroduction in the food after a prolonged mode could be made without problem. This attitude however is not commonly proposed.

See too

External bonds

French Association Of Intolerant With the Gluten
Canadian Association of the coeliaque disease
French-speaking Switzerland Association of the cœliaquie
Belgian society of Coeliaquie
Group of Studies and Research on the Coeliaque Disease of the Hospital Georges Pompidou

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