Post by kickingfrog on Feb 4, 2011 12:48:53 GMT
**********************
Primary Care Society for Gastroenterology
Coeliac Disease
Synonyms: Gluten sensitive enteropathy, Celiac Disease, Celiac Sprue
Genetically-determined chronic inflammatory intestinal disorder of the proximal bowel induced by gluten (found in wheat and barley).
Prevalance 1 in 100-200 people in the UK.
Prevalance (prospective birth cohort) studies suggest 1% of children have IgA endomysial antibodies by 7 years of age;.. although only 1:2500 will have been diagnosed as having coeliac disease at that time. ....
There must be a large number of undiagnosed coeliac patients in the community which presents a large diagnostic challenge to Primary Care.
It is a multigenetic disorder, associated with HLA types HLA-DQ2 (90%) or HLA-DQ8, plus other genetic or environmental factors. The HLA genes are not likely to be main causative factor. There is a familial tendency (10-15% of first degree relatives will also be affected), identical twins concordance is 70%.
Presentation...
May present at any age - presentation in childhood now less common, and increasingly being recognised in adults (may present with non-specific bowel or abdominal symptoms).
· Babies and Young Children present any time after weaning, (peak 9 months to 3 years). Malabsorption generally presents with diarrhoea (frequent paler stools), weight loss and failure to thrive. Vomiting, anorexia, irritability, and constipation are also common. The abdomen may protrude, with eversion of the umbilicus.
· Older children and adults may present with anaemia (folate or iron deficiency) nonspecific symptoms of abdominal discomfort, arthralgia, anaemia , fatigue and malaise, as well as diarrhoea, steatorrhoea and malabsorption Mouth ulcers and angular stomatitis are common (85% have asymptomatic iron/folate deficiency, 15-30% have Vit D deficiency, 10% Vit K deficiency). Some are identified at infertility clinics.
· Dermatitis Herpetiformis is the classic skin manifestation of coeliac disease - almost all of patients with the rash have either detectable villous atrophy (~75%) or minor mucosal changes. Acquired ichthyosis has been reported...
Histopathology Histological examination of the mucosa which has been exposed to gluten classically shows "subtotal villous atrophy" and results in malabsorption - however mucosa is of normal thickness, villous atrophy is compensated by crypt hyperplasia. There may be a range of abnormality, from a normal villous architecture with epithelial lymphocytosis, to total villous atrophy.
Investigations
· Serological testing - the preferred investigations are IgA Anti-tissue transglutaminase (tTG) or IgA Endomysial (EMA) antibodies. The former is a newer test (tTG is the autoantigen of EMA) and is gradually replacing the latter, but both are highly specific and sensitive for untreated coeliac disease provided patient is still on gluten (and the patient doesn't't have selective IgA deficiency, as occurs in ~0.4% of the general population and in 2.6% of patients with coeliac disease). Both frequently become undetectable after 6-12 months of a gluten free diet and can be used to monitor the disease.
Antigliadin antibodies are less specific, but can be either IgA (AGA) or IgG (AGG). They can be positive in other gastrointestinal conditions such as Crohn’s
· Subsequent endoscopic or enteroscopic distal duodenal or jejunal biopsy is performed (the initial investigation of choice when there is a high clinical suspicion of coeliac disease).
The diagnosis is made when biopsy shows villous atrophy while the patient is eating adequate amounts of gluten, followed by full clinical remission on excluding gluten. Under these circumstances, with tTG or EMA antibodies found at the time of diagnosis and their disappearance after gluten exclusion means that it is only necessary to perform a further biopsy (and even a further gluten challenge and more biopsies) if there are still doubts. The further gluten challenge should always be performed if coeliac disease is diagnosed in children <2 yrs because of a high incidence of other causes of flat mucosa ...
· FBC shows anaemia in 50%; iron and folate deficiency are both common (microcytes and macrocytes), hypersegmented leucocytes and Howell-Jolly bodies (splenic atrophy). Also check B12, folate, ferritin, LFT, Calcium and albumin.
· Small bowel barium studies are occasionally needed to exclude other causes of malabsorption and diarrhoea; and diagnose rare complications such as obstruction or lymphoma.
Differential Diagnosis Lactose or other food intolerances, colitis (including inflammatory bowel disease), other causes of malabsorption.
Associated conditions which occur with increased frequency in coeliac disease include osteoporosis, subfertility, and various autoimmune diseases (especially thyrotoxicosis and
and type 1 diabetes mellitus ; but is probably also true of Sjogren’s syndrome and Primary biliary cirrhosis ; and IgA deficiency
Complications Osteoporosis (even in treated cases). There is an 80x risk of bowel adenocarcinoma, and increased risk of oesophageal and oropharyngeal squamous carcinoma, intestinal T-cell lymphoma (usually presents with return of bowel symptoms and usually responds poorly to treatment), ulcerative jejuno-ileitis.....
Treatment Starting a gluten-free diet (GFD) rapidly induces clinical improvement, which is mirrored by the mucosa. The diet consists of no wheat, barley, rye, or any food containing them (eg bread, cake, pies). Moderate quantities of oats (free from other contaminating cereals) can be consumed as recent studies suggest that they do not damage the intestinal mucosa .... Rice, maize, soya, potatoes, sugar jam, syrup and treacle are all allowed. Gluten-free biscuits, flour, bread and pasta are NHS prescribable. Coeliac UK produce a prescribing guide.
Arrange dietitian appointment (with regular reviews). Even minor dietary lapses may cause recurrence. GFD should be lifelong, as relaxation of diet generally brings a return of symptoms and increased incidence of complications. Add supplements as necessary (eg folic acid, iron, calcium).
Serial tTG or EMA antibodies can be used to monitor response to diet.
Why follow-up patients with coeliac disease? ....
Patient compliance with a gluten-free diet is poor, ranging from 45-87%. The long-term health risks for patients who comply poorly with a gluten-free diet include increased risk of malignancy, nutritional deficiency and reduced bone mineral density.
· Coeliac disease is associated with an increased risk of gastrointestinal malignancy, notably lymphoma of the small intestine, although the absolute risk appears small. A strict gluten-free diet for 5 years or more protects against these malignancies.
· About a quarter of patients with coeliac disease have osteoporosis of the lumbar spine compared to 5% of matched controls. Bone mineral density improves significantly with a gluten-free diet.
· Dietary compliance positively correlates with regular follow-up and knowledge of the condition.
· Half of all coeliac patients have an inadequate energy intake, and 10% have inadequate intake of calcium and vitamin B6. 80% of elderly patients have inadequate intake of vitamin D.
· GPs are responsible for the appropriate prescription of gluten-free products.
Regular follow-up is an opportunity to provide patient centred care that is sensitive to the individual's life circumstances.
How often should patients be reviewed?
· Patients should be followed up throughout their lifetime
· After diagnosis, the patient should be reviewed at the gastroenterology clinic after 3 months and 6 months to ensure they are making satisfactory progress and managing the diet
· If well, they should be reviewed annually or sooner if problems arise - follow-up assessments are currently being carried out by dietitians, nurses, general practitioners and gastroenterologists in primary and secondary care.
What should be done at Annual Assessments? .....
Disease status:
· Weight, Height, Body Mass Index (D).
· Symptom assessment - bowel function (stool frequency, stool consistency, blood in stool) abdominal pain (D).
· Investigations: Haemoglobin. Red cell folate. Serum Ferritin. Serum Albumin. Alkaline Phosphatase. Patients with coeliac disease who adhere to a gluten-free diet often eat inadequate intakes of folic acid and iron. (B) Low haemoglobin, red cell folate, and serum ferritin may suggest persisting malabsorption warranting further assessment.
· Antibody tests can be used to monitor significant dietary gluten ingestion (C).
Disease prevention: Osteoporosis risk assessment and management
· DEXA at menopause for women, at 55 years for men, at any age following fragility fracture.
· Advise regular physical activity (A), reduce smoking and alcohol consumption (B).
· Calcium supplement - if poor dietary intake (A).
· Vitamin D supplements - if housebound (A).
· HRT and biphosphonates - if osteoporotic (A).
Some degree of splenic atrophy is present in most patients with coeliac disease, and is sufficiently severe to cause peripheral blood changes in about a quarter.
Patients should be considered for:
· Vaccination against pneumococcus and Haemophilus influenzae type b (D).
· Vaccination against influenza (D).
· Guidance about the increased risks attached to tropical infections e.g. malaria (D).
· Life long prophylactic antibiotics are not recommended (C).
Medical care: Management of associated medical problems.
· Discussion of familial risk if required. First-degree relatives of people with coeliac disease have a 1 in 10 chance of developing the disease, and should be assessed if they develop suggestive symptoms.
· Review prescription items.
Self care:
· Discuss gluten-free diet compliance and advice.
· Discuss membership of Coeliac Society.
· Discuss use of the Coeliac Society's Gluten' Free Food and Drink directory.
· Dietary advice on weight, macronutrients, calcium, vitamin D, iron and fibre intake as required.
The strength of the recommendations is based on the quality of supporting evidence:
Evidence from randomised controlled trials.
Evidence from other controlled or quasi-experimental studies.
[C] Evidence from descriptive studies.
[D] Expert opinion and clinical experience.
How should we organise the service? ....
· Create a database of coeliac patients to facilitate recall and audit. This may be organised at practice, district or primary care organisation level.
· Use a template to record clinical data in a standardised way, in order to facilitate audit and research.
· Have a named person who will have clinical and administrative responsibility for the service.
· The service should be audited annually: audit standards may include number of patients who have recorded evidence of:
o Compliance with gluten-free diet (GFD).
o Body Mass Index.
o Osteoporosis assessment.
When should the patient be under specialist care? You should consider specialist referral if there is:
· Poor response to gluten-free diet.
· Weight loss on gluten-free diet.
· Blood in stools.
· Onset of unexplained abdominal pain.
· Other clinical concerns.
Screening Coeliac disease appears to be underdiagnosed in primary care.....
In order to uncover the "iceberg" GP's need to screen for coeliac disease - especially patients with autoimmune disease, unexplained anaemia or osteoporosis; or family history of coeliac disease ....
Patients having upper GI endoscopy for iron deficiency or macrocytic anaemia may be suitable for screening with a distal duodenal biopsy if no other cause is seen. The case for childhood screening is currently being considered. NICE recommend screening children with Type 1 Diabetes for coeliac disease every 3 years until adulthood, and subsequently if adults have a low body mass index or develop unexplained weight loss. .....
www.pcsg.org.uk/
Put 'coeliac disease' in the search box.
***************************
Primary Care Society for Gastroenterology
Coeliac Disease
Synonyms: Gluten sensitive enteropathy, Celiac Disease, Celiac Sprue
Genetically-determined chronic inflammatory intestinal disorder of the proximal bowel induced by gluten (found in wheat and barley).
Prevalance 1 in 100-200 people in the UK.
Prevalance (prospective birth cohort) studies suggest 1% of children have IgA endomysial antibodies by 7 years of age;.. although only 1:2500 will have been diagnosed as having coeliac disease at that time. ....
There must be a large number of undiagnosed coeliac patients in the community which presents a large diagnostic challenge to Primary Care.
It is a multigenetic disorder, associated with HLA types HLA-DQ2 (90%) or HLA-DQ8, plus other genetic or environmental factors. The HLA genes are not likely to be main causative factor. There is a familial tendency (10-15% of first degree relatives will also be affected), identical twins concordance is 70%.
Presentation...
May present at any age - presentation in childhood now less common, and increasingly being recognised in adults (may present with non-specific bowel or abdominal symptoms).
· Babies and Young Children present any time after weaning, (peak 9 months to 3 years). Malabsorption generally presents with diarrhoea (frequent paler stools), weight loss and failure to thrive. Vomiting, anorexia, irritability, and constipation are also common. The abdomen may protrude, with eversion of the umbilicus.
· Older children and adults may present with anaemia (folate or iron deficiency) nonspecific symptoms of abdominal discomfort, arthralgia, anaemia , fatigue and malaise, as well as diarrhoea, steatorrhoea and malabsorption Mouth ulcers and angular stomatitis are common (85% have asymptomatic iron/folate deficiency, 15-30% have Vit D deficiency, 10% Vit K deficiency). Some are identified at infertility clinics.
· Dermatitis Herpetiformis is the classic skin manifestation of coeliac disease - almost all of patients with the rash have either detectable villous atrophy (~75%) or minor mucosal changes. Acquired ichthyosis has been reported...
Histopathology Histological examination of the mucosa which has been exposed to gluten classically shows "subtotal villous atrophy" and results in malabsorption - however mucosa is of normal thickness, villous atrophy is compensated by crypt hyperplasia. There may be a range of abnormality, from a normal villous architecture with epithelial lymphocytosis, to total villous atrophy.
Investigations
· Serological testing - the preferred investigations are IgA Anti-tissue transglutaminase (tTG) or IgA Endomysial (EMA) antibodies. The former is a newer test (tTG is the autoantigen of EMA) and is gradually replacing the latter, but both are highly specific and sensitive for untreated coeliac disease provided patient is still on gluten (and the patient doesn't't have selective IgA deficiency, as occurs in ~0.4% of the general population and in 2.6% of patients with coeliac disease). Both frequently become undetectable after 6-12 months of a gluten free diet and can be used to monitor the disease.
Antigliadin antibodies are less specific, but can be either IgA (AGA) or IgG (AGG). They can be positive in other gastrointestinal conditions such as Crohn’s
· Subsequent endoscopic or enteroscopic distal duodenal or jejunal biopsy is performed (the initial investigation of choice when there is a high clinical suspicion of coeliac disease).
The diagnosis is made when biopsy shows villous atrophy while the patient is eating adequate amounts of gluten, followed by full clinical remission on excluding gluten. Under these circumstances, with tTG or EMA antibodies found at the time of diagnosis and their disappearance after gluten exclusion means that it is only necessary to perform a further biopsy (and even a further gluten challenge and more biopsies) if there are still doubts. The further gluten challenge should always be performed if coeliac disease is diagnosed in children <2 yrs because of a high incidence of other causes of flat mucosa ...
· FBC shows anaemia in 50%; iron and folate deficiency are both common (microcytes and macrocytes), hypersegmented leucocytes and Howell-Jolly bodies (splenic atrophy). Also check B12, folate, ferritin, LFT, Calcium and albumin.
· Small bowel barium studies are occasionally needed to exclude other causes of malabsorption and diarrhoea; and diagnose rare complications such as obstruction or lymphoma.
Differential Diagnosis Lactose or other food intolerances, colitis (including inflammatory bowel disease), other causes of malabsorption.
Associated conditions which occur with increased frequency in coeliac disease include osteoporosis, subfertility, and various autoimmune diseases (especially thyrotoxicosis and
and type 1 diabetes mellitus ; but is probably also true of Sjogren’s syndrome and Primary biliary cirrhosis ; and IgA deficiency
Complications Osteoporosis (even in treated cases). There is an 80x risk of bowel adenocarcinoma, and increased risk of oesophageal and oropharyngeal squamous carcinoma, intestinal T-cell lymphoma (usually presents with return of bowel symptoms and usually responds poorly to treatment), ulcerative jejuno-ileitis.....
Treatment Starting a gluten-free diet (GFD) rapidly induces clinical improvement, which is mirrored by the mucosa. The diet consists of no wheat, barley, rye, or any food containing them (eg bread, cake, pies). Moderate quantities of oats (free from other contaminating cereals) can be consumed as recent studies suggest that they do not damage the intestinal mucosa .... Rice, maize, soya, potatoes, sugar jam, syrup and treacle are all allowed. Gluten-free biscuits, flour, bread and pasta are NHS prescribable. Coeliac UK produce a prescribing guide.
Arrange dietitian appointment (with regular reviews). Even minor dietary lapses may cause recurrence. GFD should be lifelong, as relaxation of diet generally brings a return of symptoms and increased incidence of complications. Add supplements as necessary (eg folic acid, iron, calcium).
Serial tTG or EMA antibodies can be used to monitor response to diet.
Why follow-up patients with coeliac disease? ....
Patient compliance with a gluten-free diet is poor, ranging from 45-87%. The long-term health risks for patients who comply poorly with a gluten-free diet include increased risk of malignancy, nutritional deficiency and reduced bone mineral density.
· Coeliac disease is associated with an increased risk of gastrointestinal malignancy, notably lymphoma of the small intestine, although the absolute risk appears small. A strict gluten-free diet for 5 years or more protects against these malignancies.
· About a quarter of patients with coeliac disease have osteoporosis of the lumbar spine compared to 5% of matched controls. Bone mineral density improves significantly with a gluten-free diet.
· Dietary compliance positively correlates with regular follow-up and knowledge of the condition.
· Half of all coeliac patients have an inadequate energy intake, and 10% have inadequate intake of calcium and vitamin B6. 80% of elderly patients have inadequate intake of vitamin D.
· GPs are responsible for the appropriate prescription of gluten-free products.
Regular follow-up is an opportunity to provide patient centred care that is sensitive to the individual's life circumstances.
How often should patients be reviewed?
· Patients should be followed up throughout their lifetime
· After diagnosis, the patient should be reviewed at the gastroenterology clinic after 3 months and 6 months to ensure they are making satisfactory progress and managing the diet
· If well, they should be reviewed annually or sooner if problems arise - follow-up assessments are currently being carried out by dietitians, nurses, general practitioners and gastroenterologists in primary and secondary care.
What should be done at Annual Assessments? .....
Disease status:
· Weight, Height, Body Mass Index (D).
· Symptom assessment - bowel function (stool frequency, stool consistency, blood in stool) abdominal pain (D).
· Investigations: Haemoglobin. Red cell folate. Serum Ferritin. Serum Albumin. Alkaline Phosphatase. Patients with coeliac disease who adhere to a gluten-free diet often eat inadequate intakes of folic acid and iron. (B) Low haemoglobin, red cell folate, and serum ferritin may suggest persisting malabsorption warranting further assessment.
· Antibody tests can be used to monitor significant dietary gluten ingestion (C).
Disease prevention: Osteoporosis risk assessment and management
· DEXA at menopause for women, at 55 years for men, at any age following fragility fracture.
· Advise regular physical activity (A), reduce smoking and alcohol consumption (B).
· Calcium supplement - if poor dietary intake (A).
· Vitamin D supplements - if housebound (A).
· HRT and biphosphonates - if osteoporotic (A).
Some degree of splenic atrophy is present in most patients with coeliac disease, and is sufficiently severe to cause peripheral blood changes in about a quarter.
Patients should be considered for:
· Vaccination against pneumococcus and Haemophilus influenzae type b (D).
· Vaccination against influenza (D).
· Guidance about the increased risks attached to tropical infections e.g. malaria (D).
· Life long prophylactic antibiotics are not recommended (C).
Medical care: Management of associated medical problems.
· Discussion of familial risk if required. First-degree relatives of people with coeliac disease have a 1 in 10 chance of developing the disease, and should be assessed if they develop suggestive symptoms.
· Review prescription items.
Self care:
· Discuss gluten-free diet compliance and advice.
· Discuss membership of Coeliac Society.
· Discuss use of the Coeliac Society's Gluten' Free Food and Drink directory.
· Dietary advice on weight, macronutrients, calcium, vitamin D, iron and fibre intake as required.
The strength of the recommendations is based on the quality of supporting evidence:
Evidence from randomised controlled trials.
Evidence from other controlled or quasi-experimental studies.
[C] Evidence from descriptive studies.
[D] Expert opinion and clinical experience.
How should we organise the service? ....
· Create a database of coeliac patients to facilitate recall and audit. This may be organised at practice, district or primary care organisation level.
· Use a template to record clinical data in a standardised way, in order to facilitate audit and research.
· Have a named person who will have clinical and administrative responsibility for the service.
· The service should be audited annually: audit standards may include number of patients who have recorded evidence of:
o Compliance with gluten-free diet (GFD).
o Body Mass Index.
o Osteoporosis assessment.
When should the patient be under specialist care? You should consider specialist referral if there is:
· Poor response to gluten-free diet.
· Weight loss on gluten-free diet.
· Blood in stools.
· Onset of unexplained abdominal pain.
· Other clinical concerns.
Screening Coeliac disease appears to be underdiagnosed in primary care.....
In order to uncover the "iceberg" GP's need to screen for coeliac disease - especially patients with autoimmune disease, unexplained anaemia or osteoporosis; or family history of coeliac disease ....
Patients having upper GI endoscopy for iron deficiency or macrocytic anaemia may be suitable for screening with a distal duodenal biopsy if no other cause is seen. The case for childhood screening is currently being considered. NICE recommend screening children with Type 1 Diabetes for coeliac disease every 3 years until adulthood, and subsequently if adults have a low body mass index or develop unexplained weight loss. .....
www.pcsg.org.uk/
Put 'coeliac disease' in the search box.
***************************