Celiac Disease Abstracts Description, Incidence & Genetics

Abstract Patients with coeliac disease are at increased risk of developing complications which increase morbidity and mortality. Emphasis on malignant complications has often overshadowed the non-malignant risks, which have received relatively little attention, although some of these can be very troublesome and even life-threatening. This article points out that a large population of unidentified or neglected coeliac patients is at potential risk. The challenge is to identify this group by case-finding or screening programmes in selected populations, so that they can be offered a gluten-free diet and other treatments which will not only improve general health but may also prevent or reduce the development of health problems. The non-malignant risks are outlined and bone and neuropsychiatric disturbances considered in more detail because of recent developments in these areas.

Coeliac disease research and clinical practice: maintaining momentum into the twenty-first century.

Author Ferguson A

Address University of Edinburgh, UK.

Source Baillieres Clin Gastroenterol, 1995 Jun, 9:2, 395-412

Abstract Recent research shows that each word in the definition of coeliac disease, permanent gluten sensitive enteropathy, must now be reviewed, revised or reinterpreted. Permanent--but there are now well documented cases of acquired disease, and perhaps also partial recovery of gut gluten tolerance. Enteropathy--gluten sensitivity is expressed in a spectrum, with a mild form seen as normal architecture with high count of intraepithelial lymphocytes. Gluten--the provoking agent--Investigators are intensively working to identify the precise toxic sequence, and to establish how this will link in with new genetic information. Mechanism of sensitivity? or hypersensitivity?--Critical to this is new knowledge on the modulation and regulation of immunity to intestinal antigens, including gliadin. A hypothesis is presented, as to the pathogenesis of gluten-sensitive enteropathy, which combines concepts of oral tolerance and of the regulation of expression of delayed type hypersensitivity reactions in the gut mucosa.

How many hospital visits does it take before celiac sprue is diagnosed?

Author Dickey W; McConnell JB

Address Department of Gastroenterology, Antrim Hospital, Northern Ireland.

Source J Clin Gastroenterol, 1996 Jul, 23:1, 21-3

Abstract We studied the hospital records of patients with celiac sprue in order to determine how frequently hospital specialists failed to make the diagnosis. Over a 7 1/2-year period, 39 patients were diagnosed, 49% within the last 18 months of the study period. Fourteen patients (39%) had been referred to the hospital a total of 30 times with features suggestive of celiac sprue, yet without being successfully diagnosed: the delay between initial referral and diagnosis was 6 years in nine of these patients. The diagnosis was made by gastroenterologists or other internists in 38 (97%) patients. Gastroenterologists had an 85% (33 of 39) diagnostic success rate, other internists 63% (five of eight), and surgeons 7% (one of 14). None of eight referrals to other specialists led to diagnosis. While a history of diarrhea was morel likely to lead to diagnosis, it was reported by only 59% (23 of 39) of patients at the time of diagnosis and at only 46% (32 of 69) of referrals; furthermore, it did not prompt correct diagnosis in 28% (nine of 32). Anemia was the sole manifestation of celiac sprue at 17 referrals, and correct diagnosis was made in only seven (41%), all by gastroenterologists. The perceived rarity of celiac sprue reflects its underdiagnosis. Diagnosis is still delayed even in patients with classic diarrhea, and there is still a failure to appreciate the possible manifestations of sprue, including anemia without gastrointestinal symptoms. Because patients may be referred to specialists other than gastroenterologists with symptoms arising from celiac sprue, a wider knowledge of its manifestations is called for.

The coeliac disease task force " Free from Gluten," " Improved knowledge to cure coeliac disease".

Author Greco L; Percopo S

Address Department of Pediatrics, University Federico II, Naples, Italy.

Source Acta Paediatr Suppl, 1996 May, 412:, 25-8

Abstract Gluten has recently been introduced into the diet of the Mediterranean and European population, but a considerable proportion has not adapted to this change and has developed intolerance to gluten. At least one million EEC citizens are gluten intolerant. In Italy each year 12 million ECU is spent in the diagnosis of uncomplicated gluten intolerance, 10 million ECU for complex diagnosis and 32 million ECU for long-term complications and malignancies. The total financial load of gluten intolerance, in its present state, is about 150 million ECU/year in Italy. Four million ECU is actually needed to develop a genetic probe, which may make the population screening feasible. A National Task Force "Free from Gluten" has been set up by the Italian Coeliac Society to stimulate fund-raising activities supporting genetic and basic research on gluten intolerance.

Striking differences in the incidence of childhood celiac disease between Denmark and Sweden: a plausible explanation.

Author Weile B; Cavell B; Nivenius K; Krasilnikoff PA

Address Department of Paediatrics, University of Copenhagen, Gentofte Hospital, Denmark.

Source J Pediatr Gastroenterol Nutr, 1995 Jul, 21:1, 64-8

Abstract Among 771 children (381 Swedish and 390 Danish) investigated between 1972 and 1989 because of suspected celiac disease (CD), 179 proved to have the disease. Surprisingly only 24 CD patients were found among the Danish children, compared with 155 in the Swedish group, despite the close ethnic, geographical, and cultural background of the two populations. The Swedish CD children were diagnosed at an earlier age than the Danish children (mean, 1.5 vs. 5.5 years). The symptoms of the Swedish patients were dominated by failure to thrive (93 vs. 71%), whereas a higher proportion of the Danish CD patients suffered from stomach pain (21 vs. 5%). Breast-feeding habits were comparable. The estimated content of gliadin in the officially recommended diets of the two countries in 1987 differed substantially, the Swedish diet containing more than 40 times more gliadin than the Danish (4,400 vs. 100 mg) at the age of 8 months, and 4 times more (3,600 vs. 900 mg) at the age of 12 months. The Danish infant diet differed significantly from the Swedish in containing a larger amount of the lower gluten-containing rye flour. The earlier introduction of food items with a high gluten content in the Swedish compared with the Danish diet seems to be an obvious explanation for the great difference in incidence and symptomatology of CD between the two populations.

Coeliac disease in adults.

Author Corazza GR; Gasbarrini G

Address University of L'Aquila, Italy.

Source Baillieres Clin Gastroenterol, 1995 Jun, 9:2, 329-50

Abstract Coeliac disease is a chronic disease characterized by small bowel villous atrophy which impairs nutrient absorption and improves on withdrawal of wheat gliadins and barley, rye and oat prolamins from the diet. Knowledge of the adult form of coeliac disease has greatly improved in recent years. Although this knowledge is not yet sufficiently widespread among referring clinicians, it has, over the past few years, allowed an increasing number of patients to be diagnosed with subclinical forms characterized by minor, transient or apparently unrelated symptoms. As a consequence, our views on the clinical and epidemiological aspects of this condition, the prevalence of which in the general population is believed to be close to 1 in 300, have changed and are still changing. Since it has been demonstrated that a strict gluten-free diet is protective against the complications of adult coeliac disease, it is important that even subclinical and silent forms are diagnosed and treated as early as possible. Non-invasive screening tests, such as anti-gliadin and anti-endomysium antibody estimation, should therefore be used systematically in groups considered to be at risk of coeliac disease. These include first-degree relatives of coeliac patients and patients with insulin-dependent diabetes mellitus, iron-deficiency anaemia, epilepsy with cerebral calcification, recurrent aphthous stomatitis and dental enamel hypoplasia. Other conditions will probably be identified in the near future.

Coeliac disease in childhood.

Author Littlewood JM

Address St. James's University Hospital, Leeds, UK.

Source Baillieres Clin Gastroenterol, 1995 Jun, 9:2, 295-327

Abstract Coeliac disease usually presents in infancy or early childhood with diarrhoea, vomiting and interference with weight gain and growth. Withdrawal of dietary gluten is followed by resolution of the symptoms and signs and restoration of normal weight gain and growth; the characteristic subtotal villous atrophy of the jejunal mucosa also recovers. Later re-introduction of dietary gluten will lead to a return of the jejunal mucosal abnormality in the majority and to clinical relapse in many but not all. The severity and timing of both are variable and 5% of children initially considered on clinical, biopsy and gluten response evidence to have coeliac disease appear to develop permanent tolerance to gluten, although mucosal relapse may occur years after the re-introduction of dietary gluten in a minority, emphasizing the need for long-term follow-up. Although a diagnostic and subsequent follow-up jejunal biopsy are necessary to confirm the diagnosis, anti-gliadin IgA and IgG, anti-reticulum and anti-endomysium antibodies are now almost totally reliable in identifying children who have coeliac disease and are valuable in monitoring the adequacy of gluten withdrawal. Dietary compliance is frequently poor and regular supervision by a paediatric dietitian is needed; indeed, lifelong supervision to ensure gluten withdrawal is essential to reduce the chance of developing later gastrointestinal malignancy.

Gender and clinical presentation in adult celiac disease.

Author Ciacci C; Cirillo M; Sollazzo R; Savino G; Sabbatini F; Mazzacca G

Address Gastrointestinal Unit, Medical School, University Federico II of Naples, Italy.

Source Scand J Gastroenterol, 1995 Nov, 30:11, 1077-81

Abstract BACKGROUND: Celiac disease may present in various forms. This study aimed to investigate whether gender affects the clinical presentation of the disease in adult celiac patients from the Mediterranean area. METHODS: This study retrospectively analyzes data collected in all adult patients with celiac disease (n = 195) seen during the past 13 years at the Gastrointestinal Unit of the Federico II University of Naples, Italy. RESULTS: In these series of patients the ratio of women to men was 3.33. Age at diagnosis was lower in women that in men (p < 0.05). Except for asthenia, all signs and symptoms were more frequent in women than in men. Hypochromic anemia was the most commonest finding in women and was 40% more frequent in women than in men (p < 0.001). Dyspepsia was twice as frequent in women as in men (p < 0.05); genital disorders were reported by 44% of women and by no men. Recent weight loss or low body mass index was the commonest finding in men. About 60% of men and women reported diarrhea; among patients without diarrhea, the prevalence of hypochromic anemia differed between sexes (p < 0.05), occurring in about 80% of women. CONCLUSION: This study shows that the clinical presentation of celiac disease is not the same in men and women. The disease is not only more frequent in women than in men but is also more severe and more rapid. The data also suggest the need to look for celiac disease in patients with unexplained hypochromic anemia.

High prevalence of undiagnosed coeliac disease in 5280 Italian students screened by antigliadin antibodies.

Author Catassi C; R?tsch IM; Fabiani E; Ricci S; Bordicchia F; Pierdomenico R; Giorgi PL

Address University Department of Paediatrics, Ancona, Italy.

Source Acta Paediatr, 1995 Jun, 84:6, 672-6

Abstract Many cases of coeliac disease are currently undiagnosed. We carried out a pilot study on screening for coeliac disease in a school population. The screening protocol consisted of three parts: (1) IgG and IgA antigliadin antibody (AGA) assay; (2) antiendomysium antibody and total serum IgA determinations; (3) jejunal biopsy. A total of 5280 students aged 11-15 years (71.7% of the eligible population) underwent the first evaluation; 113 subjects performed the second tests and 35 of these needed the third investigation. Coeliac disease was diagnosed in 23 cases, most of which were atypical or silent forms. The prevalence of undiagnosed coeliac disease was 4.36 per 1000 screened subjects (95% CI 2.58-6.14) and 5.03 per 1000 (95% CI 3.41-6.65) in the general population. The ratio of known to undiagnosed cases was 1 to 6.4. This high prevalence of undiagnosed coeliac disease raises a number of problems that require further evaluation.

Prevalence and diagnosis of celiac disease in IgA-deficient children.

Author Meini A; Pillan NM; Villanacci V; Monafo V; Ugazio AG; Plebani A

Address Department of Pediatrics, University of Brescia, Italy.

Source Ann Allergy Asthma Immunol, 1996 Oct, 77:4, 333-6

Abstract BACKGROUND: Reported frequencies of celiac disease in selective IgA deficiency in childhood vary widely and this is probably due to the different characteristics of the patients studied and to the different criteria used for intestinal biopsy: all patients or only those with symptoms of malabsorption. Diagnosis of celiac disease is of considerable importance in IgA deficiency because of its increased frequency and also because avoidance of dietary gluten permits elimination of the symptoms and complications of celiac disease. OBJECTIVES: To obtain a more reliable estimate of the incidence of celiac disease in childhood IgA deficiency jejunal biopsies were performed in 65 consecutively diagnosed IgA-deficient children whose parents consented. Some clinical and laboratory parameters including IgA-antigliadin and IgG-antigliadin antibodies were evaluated to predict their usefulness in selecting IgA-deficient patients for intestinal biopsy. METHODS: All IgA-deficient patients had serum IgA levels below 5 mg/dL and salivary IgA below 0.5 mg/dL. Jejunal biopsy was performed using a peroral Watson capsule and IgA-antigliadin and IgG-antigliadin antibodies were performed by an ELISA assay. RESULTS: Biopsy findings of severe villous atrophy permitted diagnosis of celiac disease in 7.7% (5/65 children). IgG-antigliadin antibody levels, elevated in 16 patients including all five celiacs, were the best parameter for predicting celiac disease and gave no false negatives. CONCLUSIONS: The 7.7% frequency of celiac disease observed in these IgA-deficient children is about 20 times higher than in the general Italian population, and the lowest among the studies biopsying all patients; this is probably attributable to the presence of a substantial proportion of healthy children (20/65) and very few (2/65) with autoimmune disorders. The elevated sensitivity and negative-predictive value of IgG-antigliadin antibodies lead us to suggest that positive IgG-antigliadin antibodies can be used to select IgA-deficient children for jejunal biopsy with a very low probability of missing celiac disease while allowing a drastic reduction in the number of biopsies performed.

Is celiac disease a lifelong disorder?

Author Schmitz J

Address Department of Pediatrics, H?pital des Enfants Malades, Paris.

Source Clin Invest Med, 1996 Oct, 19:5, 352-6

Abstract That celiac disease is a lifelong disorder was suggested by clinical case records and was considered to have been demonstrated through the widespread use of intestinal biopsies by the end of the 1950s. It was clear that the mucosal lesions observed in children and adults were identical and responded similarly to gluten withdrawal. In fact, in 1970 the European Society for Paediatric Gastroenterology and Nutrition instituted the practice of a challenge after diagnosis. A relapse of clinical symptoms and of the intestinal lesions after gluten was reintroduced into the diet demonstrated the "permanent" nature of sensitivity to gluten in children with celiac disease. Twenty-five years later, the permanence of the sensitivity of the intestinal mucosa to gluten is again a matter of debate. Several lines of evidence, gathered during recent years, show that celiac disease is not always a lifelong condition. First, the long-term follow-up of children with proven celiac disease shows that 10% to 20% of them become "tolerant" (defined on clinical, biological and histologic grounds) to gluten during adolescence. Second, it has also been shown, in individual cases, that the mucosal lesions typical of the disease may appear during adulthood. Our increasing knowledge of the long-term evolution of the disease suggests that celiac disease develops and, in some cases, fades in a predisposed group of people with intestinal sensitivity to gluten, which is probably a common condition. The factors leading to the appearance or disappearance of the disease, however, are still unknown.

Celiac disease is a lifelong disorder.

Author Chartrand LJ; Seidman EG

Address Division of Pediatric Gastroenterology, Sainte-Justine Hospital, Quebec.

Source Clin Invest Med, 1996 Oct, 19:5, 357-61

Abstract Celiac disease has always been considered a permanent condition. A relapse, defined on the basis of mucosal changes, occurring within 2 years of reintroducing gluten to a patient's diet (challenge) has been taken as confirmation of the permanence of the disease. Some observers have questioned whether the disease is permanent, since long periods of unexplained clinical remission occur, mainly among adolescents. However, the presence or absence of symptoms has no correlation with the histologic activity of the disease or with the results of serologic tests. With very few exceptions, patients in whom celiac disease is diagnosed during their childhood eventually have a relapse. However, in some cases, many years may elapse before a relapse; therefore, the 2-year limitation is no longer considered valid. On the other hand, there have been anecdotal observations that some patients eating a normal diet containing gluten appear to have experienced a "natural recovery." This recovery is partial and probably temporary, since there is evidence that celiac disease can be present in a latent form. Long-term randomized studies, in which morphometric and ultrastructural measurements are taken, that show villous integrity, the absence of abnormal inflammation and a lack of long-term complications of a diet containing gluten are needed before the current "zero-gluten" approach to celiac disease is altered. The individual variation in the extent and time course of celiac disease does not contradict the evidence that the disease persists throughout life, actively, silently or latently. Currently, there is no justification for recommending long-term consumption of gluten for either children or adults with celiac disease.

A comparison of diets with and without oats in adults with celiac disease

Author Janatuinen EK; Pikkarainen PH; Kemppainen TA; Kosma VM; J?rvinen RM; Uusitupa MI; Julkunen RJ

Address Department of Medicine, Kuopio University Hospital, Finland.

Source N Engl J Med, 1995 Oct 19, 333:16, 1033-7

Abstract

BACKGROUND. Wheat, rye, and barley damage the small-intestinal mucosa of patients with celiac disease; maize and rice are harmless. The effects of a diet containing oats are uncertain. METHODS. In a randomized trial, we compared the effects of gluten-free diets without oats and with oats (with a goal of 50 to 70 g per day from three sources: two types of wheat-starch flour mixed with an equal amount of oats, muesli containing 60 percent oats, and rolled-oat breakfast cereal). Fifty-two adults with celiac disease in remission were followed for 6 months and 40 with newly diagnosed disease for 12 months. Endoscopy with duodenal biopsy was performed at the beginning and end of the study. RESULTS. The mean (+/- SD) oat intake in the oat group was 49.9 +/- 14.7 g per day at 6 months for patients in remission and 46.6 +/- 13.3 g per day at 12 months for patients with newly diagnosed disease. The oat and control groups did not differ significantly in nutritional status, symptoms, or laboratory measures. Patients in remission, regardless of diet, did not have worsening architecture of the duodenal villi or increased mononuclear-cell infiltration. All the patients with new diagnoses were in remission at one year, except for one in the control group. Six patients in the oat groups and five in the control group withdrew from the study. CONCLUSIONS. Moderate amounts of oats can be included in a gluten-free diet for most adult patients with celiac disease without adverse effects.

Genetics

HLA genotypes and the increased incidence of coeliac disease in Sweden.

Author Ploski R; Ascher H; Sollid LM

Address Institute of Transplantation Immunology, National Hospital, University of Oslo, Norway.

Source Scand J Gastroenterol, 1996 Nov, 31:11, 1092-7

Abstract BACKGROUND: A strong increase of childhood coeliac disease (CD) was found in Sweden concurrently with changes in the infant feeding pattern. We investigated whether this increase reflects a recruitment of individuals with less predisposing HLA genotypes. METHODS: Genomic HLA-DRB1, -DQA1, and -DQB1 typing was performed in 135 Swedish patients (48 belonging to a low- and 81 to a high-incidence cohort) and 179 controls. The distribution of HLA class-II genotypes in the cohorts was compared. RESULTS: DQA1*0501 and DQB1*02 conferred increased risk for CD, and a gene dosage effect of DQB1*02 was found. The distribution of HLA genotypes among the cohorts did not differ. CONCLUSIONS: The results suggest that Swedish CD patients do not differ in genetic susceptibility compared with other populations. No evidence was found suggesting that the increase would be a result of more frequent development of disease in individuals carrying less predisposing HLA genotypes.

Recommendations Alpha Nutrition is gluten-free and is recommended as the diet revision strategy for anyone with diagnosed celiac disease, or any person with symptoms suggestive of gluten allergy.

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