In females than males with ratio between 1.5

In accordance withthe NICE guidance Coeliac disease is suspected with any patient attended healthcare personnel with Persistent abdominalpain weight loss/ faltering growth, unexplained Iron and B12 deficiency andsymptoms like Irritable bowel syndrome. Miss B, when initially seen GP, wasconcerned of general fatigue and weight loss.

Her blood tests showed Irondeficiency anaemia and B12 deficiency. According to the guideline, Coeliac screening should have been offered at that time. What is coeliac disease?How common is it? Coeliac disease is a condition where there ispermanent gluten intolerance. It is an immune-mediatedsmall intestinal enteropathy triggered by exposure to gluten. Although prevalencevaries considerably, a large-scale screening study in UK Germany Italy and Finlandshowed prevalence of 1%(ref 1-3)It is most commonly presented females than males withratio between 1.

5 and 2. (ref 1-3)For ages, it wasthought CD mainly presents in paediatric population but later studies andsystemic reviews showed that it can present at any age.   Why Coeliac disease suspected in a patient with vague abdominal symptoms andunexplained Iron and B12 deficiency? Pathophysiology: Glutenis a storage protein in wheat rye barley and possibly oats. In people who have the predisposition to CD when exposed to gluten, it activates T cells in the smallintestine which results in the release ofinflammatory mediators.

This, in turn, damages the chorionic villi inintestines causing malabsorption and other gastrointestinal symptoms.  Clinical presentation: Traditionally patients presented with malabsorptionweight loss and failure to thrive. This is known as a classical triad. Recently it was noted more varied clinicalpresentations associated with CD. Newly diagnosed patients can present with a wide range of symptoms and signs including anaemia, vague abdominal symptoms (oftenlike IBS) short stature, ataxia, depression, osteomalacia,osteoporosis, liver disease and lymphomas (ref 5). Dermatitisherpetiformis is a cutaneous manifestation of CD.It is characterized by herpetiform clusters ofintensely itchy urticated papules and small blisters distributed on theextensor aspects of the elbows and knees and over the buttocks and on thescalp. This is commonly noted in the third and fourth decade, though thecondition may occur at any age after weaning.

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Male patients are affected twiceas often as female patients. For most patients, the disease is lifelong withvarying periods of activity, potentially due to varying degrees of dietaryadherence. Miss B had general fatigueand weight loss initially.

Her BMI at initial presentation was low. She Hadbloating and mouth ulcers when she visited the GP for the second time. These symptoms suggestA possible intestinal pathology. Most common intestinal pathology with vitaminand iron deficiency is Coeliac disease. Miss B had coeliac screeningwhich was positive for the Coeliac disease.In accordance with the Coeliac screen, she had been tested for IgATTG.

As this was weakly positive IgG She had been tested for IgA EMA which waspositive.How do we diagnose Coeliacdisease? What tests are available? Investigation:  Diagnosis of CD based on serology and intestinalbiopsy when the person is on a normal gluten-containingdiet. Biopsy remains essential for adult patients and cannot be replacedby serology.

 Serological tests involve testing for the presence of specific endomysial antibodies (EMAIg A) and anti-tissue transglutaminase antibodies (IgA TTG) in the serum. Thosewho have an associated IgA deficiency IgG TTG is primarily useful (Level 1evidence). Hence while doing serological tests for Coeliac disease total IgA levels should also be checked.

 Miss B had been referred to gastroenterologistappropriately according to NICE guidance. She underwent Intestinal Biopsy whichconfirmed the diagnosis.  ESPGHAN recently produced guideline on the diagnosis of CD in children and suggested thatSymptomatic children with IgA TTG2 level more than 10 times normal limits andEMA antibodies also positive on a blood sample separately taken with positivegenetics do not need biopsies. NICE guidelines suggest genetic testing onlyunder exceptional circumstances like children who cannot have a biopsy or in people who already on limitedgluten ingestion and choose not to have gluten challenge.But in the adultpopulation, it is still recommended that biopsy is the gold standard fordiagnosis.

 Dermatitis Herpetiformis isdiagnosed by the presence of granular IgA deposits in the dermal papillae ofuninvolved peri-lesional skin as shown by direct immunofluorescence. The diagnosisshould not be made unless this has been confirmed.Conditions associated with coeliac disease areEpilepsy Type 1 IDDM, Down syndrome How do we manage Coeliac disease? GlutenFree diet for lifelong is essential inthe management of coeliac disease. Withinfew days, many see significantimprovement. Full recovery of villi in histology takes up to 2-3 months. There is level 2 evidencethat a GFD improves body weight, body mass index and bone mineralization symptomaticadults and children with CD.  While the term “gluten-free”implies the complete elimination of allsources of gluten this is not possible due to contamination of foods with traceamounts of gluten. Hence the term “gluten-free”indicates a diet that contains gluten at such a low level as to be consideredharmless.

The exact level below whichgluten is harmless is not known, but a recent review suggests less than 10 mgper day is unlikely to cause damage in most patients. (ref 9) Failure to adhere to the GFD carries a risk for adverse health consequences and increased mortality. Malignancieslike, small-bowel adenocarcinoma, cancer of the oesophagus,B-cell and T-cell non-Hodgkin lymphomas, and intestinal lymphomas ate morecommon in patients with untreated CD. Evidence suggests the risk for increasedmortality and malignancies is reduced in those who adhere to the diet.  Women with CD have an increased risk of infertility, spontaneousabortions, preterm deliveries, and delivery of low birth weight infants.Treatment of women with CD with GFD reduces these risks to that of the generalpopulation.

 Can Miss B consume Oats? Consumption of oats improves the nutrient content of the dietsof people on a GFD by increasing the intake of fibre,vitamin B, magnesium, and iron. While in the past there has been concern thatoats can cause intestinal mucosal damage in people with CD, recent evidencesuggests oats that are pure and uncontaminated by other gluten-containinggrains can be safely ingested by most people with CD provided they are taken inlimited quantities.  However, there isstill need for caution when introducing oats into the diet of people with CD asthere is a high likelihood that commercial oats may be contaminated with glutenfrom other grains.  There is alsoevidence that a small number of people with CD may be intolerant to pure oatsand can develop an immunological response to Oat Avenins. Even if confirmed tobe pure, if oats are introduced into the diet of people with CD there should bea careful follow-up to monitor for signsof both clinical and serological relapse (ref 9) NICEguideline also suggests that gluten-freeoats can be introduced at any point and will be monitored for ant immunologicalor serological response.  Although ithas also been suggested that the acceptable threshold for gluten content ingluten-free products can from the clinical point of view be set at 100 ppm (¼mg/kg), the Codex Alimentarius Commission of the WHO issued new guidelinesfor gluten content of processed food in 2008 and a law from the EuropeanCommission (EC41/2009), effective since January 2012, stipulated that foods labelledas ‘gluten-free’ should contain ?20 parts per million of gluten, and that this gluten content issafe for the coeliac population.

The20ppm threshold for gluten-free food is also accepted by the US Food and DrugAdministration, effective since August 2013. (ref 5) A mostcomprehensive systematic review suggested a daily gluten intake of <10mh is unlikelyto cause significant histological abnormalities. (level 2 evidence) Following a GFD can be cumbersome and strict avoidance of glutenis difficult because there are many hidden sources of gluten in commercial food products. There is evidence that compliancewith the GFD is improved in those who are more knowledgeable about CD and thediet.

Registered dietitians are trained to evaluate patients for potentialcurrent and future dietary nutrient deficiencies and advise and educate them onhow to maintain a strict GFD with the provisionof healthy alternatives to gluten. New technologies sometimes can be a hazard for people withCoeliac disease. Coating the fresh fruits like an apple with gluten to make the shine is the best example of it.Coeliac UK regularly works with food manufacturers and to makesure food labels are appropriate and produce an updatedlist of gluten-free products. Monitoring CD:Adherence to a gluten-free diet is the most important factor for the health of CD patient. There is noevidence available to grade a recommendation to monitor the adherence.Monitoring the patients regularly in the clinic with appropriate dieticiansupport as well as patient and family education play an important role inManagement of CDAn observational study showedimprovement with a regular follows upthan without follow up. Factors contributing to adherence are:1.

     Availability of gluten-free diet2.     Patient/ carer understanding ofthe disease3.     Financial, Social andPsychological support available in communityNICE guideline suggests an annual review of all patients to monitor weightHeight, review of symptoms, advice on gluten-freediet and identifying ant any micronutrient deficiencies and treating them. The Dietician should provide allthe information regarding gluten-freediet and suitable alternatives. Explanations of food labelling and informationon sources of a gluten-free diet shouldbe provided. Managing social situations like eating out and travelling awayfrom home can be quite difficult.

Appropriate support should be provided.Information regarding national and regional coeliac groups all patients andcarers should be provided and encourage them to join in them. (Ref 8) Miss B was noted to have anaemiaand low Vitamin B12 levels required iron and vitamin supplement.

 There is some evidence that people with untreated CD are morefrequently deficient in many micronutrients compared to those without CD.Micronutrient deficiencies identified include iron, folic acid, and vitamin B12and B6. Low bone mineral density in people with untreated CD is believed to bepartly due to vitamin D deficiency. Other deficiencies described in CD includecopper, zinc, and carnitine. Monitoring these micronutrients at the time ofdiagnosis as well later in follow up is necessary. The risk of osteoporosis and bone fracture is increased with CD.A British study showed an excess of 320/100000persons with osteoporosis.

Hencea separately designated clinic with adietician support is required. BSG encourages to measure bone density in high-risk groups and over 55yrs. (Ref 5) Hyposplenism is associated with CD. This may result in impairedimmunity to encapsulated bacteria. Vaccination against pneumococcus isrecommended. Conditions associated with the coeliac disease are Epilepsy Type 1 IDDM, Down syndrome. Patientsshould be screened and monitored for the above in their annual follow up.

 Financial aspect: Miss B recently lost her job.While she is looking for another job, she is finding it difficult to buy gluten-free diet from the supermarket. Shediscussed with the local pharmacist when she came to collect her medication.

  Gluten-free products areavailable by prescription. They are freefor children under 16, 16-18 with full-timeeducation, and adults over 60 yrs. Income support and pregnant women alsoqualify for a free prescription. Prepaidprescription service available in many CCG’s. This costs cheaper.

PharmacistsGPS and Dieticians should be able to providethe information regarding this service.  Quality of life (Psychosocial impact)Several studies showed that patients with CD have low Quality oflife than the general population.Coeliac disease can have animpact on psychological health as a biologicalimbalance due to malabsorption can,in turn, cause emotional problems. A new research has shown that it can manifest itself through psychological problemsimpacting Cognition, Emotions, Behaviour and social interactions.Persons with CD may experience anxiety and low mood(depression). Recent metanalysis showed Depression is more common in patientswith CD. Early recognition of these symptoms and managing them with appropriatehealth care support if necessary.

 A significant number of coeliacs in Europe and North Americahave reported difficulties following GFD especially in restaurants, socialfunctions and while travelling (bj). A survey shows that chefs’ knowledge aboutCD is lower than that of the general public. Therefore, education about a GFDneeds to be directed to catering personnel. A study in 2012 on 106 women >20yrs age showed that patient’sknowledge of their own problem improvesphysiological and psychological outcomes. In this study, the interventional group (54) underwent a 10sessioneducational program called Coeliac school reported asignificant improvement in psychological well-being at 10 weeks, whereas thecontrols (52) given usual care reported a worsening in psychologicalwell-being. A significant improvement remained even after 6 months.

(ref7) Studies have shown that connecting with others enhancespeople’s ability to handle gluten-freediet. Counselling should be offered wherever necessary. Pharmacists can involvein this activelyas they are more likely to see them regularly. Pele also encouraged to joinlocal Coeliac groups. What will be the chances of Miss B continuing to suffer from the same symptoms after changing on to gluten-free diet? Non-responsive CDAfteradoption of the GFD 4-30% of patients with CD report persisting symptoms and areaffected by non-responsive CD (ref 5). Inadvertent or deliberate glutenexposure is a suggested reason for NRCD. Potential complications or coexistingconditions (such as Irritable bowel syndrome, lactose intolerance, bacterialcolitis and inflammatory colitis) may be one of the reasons for persistingsymptoms.

Specialist referral required, and prednisolone can be consideredwhile awaiting specialist referral. Clinicians Dieticians and pharmacistsshould be aware of other differentials that can present similarly to the Coeliac disease. Conditions other than coeliac disease that can causedestruction of villi are 1.     Cows’ milk protein intolerance 2.     soya protein intolerance, 3.

     Gastroenteritis, 4.     Zollinger Ellison syndrome (a syndrome caused bynon-beta cell tumour of pancreatic islets), 5.     tropical Sprue If Miss B Still continue to have symptomsdespite strictly on GFD reconsideration of the diagnosis is reasonable.

Otherintestinal pathologies may present with similar symptoms as abdominal pain and weight loss. Inflammatory bowel disease canpresent with abdominal pain and diarrhoea(sometimes blood in stools). Investigations looking for inflammatory markers(ESR, CRP) would help. Intestinal biopsy findings are different from Coeliacdisease.  Doany of the other family members need to be tested for the Coeliac screen?MissB’s immediate family members ideally are advised to get tested as this can runin families.

 Screeningfor CDThere is insufficient evidence to recommend population screeningfor CD, however, there should be a lowthreshold for case finding in clinical practice as per National Institute forHealth and Care Excellence guidelines. British society of gastroenterologists suggested that symptomatic first-degree relatives of patients with CDshould undergo CD testing.  What can other health professionalsdo in the management of coeliac disease? Pharmacists can play a significantrole in identifying potential cases of coeliac disease. Patientsconsulting pharmacists for recurrent diarrhoeashould be referred to their GP. By raising awareness and helping people with early diagnosis and supporting those whowere diagnosed pharmacists can improvethe quality of life and reduce chronic problems.

 In Scotland, aCentralised Pharmacist-led Scheme isintroduced which helps patients prescribing gluten-freediet as well as supporting and monitoring compliance. This has helped toimprove the service much better. Recent advances: Research looking into any other medication to reduce the immunologicalresponse is going on. So far a few medications like xxxx have shown some benefitin addition to Gluten free diet. More evidence is required to go further. Conclusion: Coeliac disease is suspected when a person presentswith Loss of weight altered bowel habits. Diagnosis involves serological andgenetic test and confirmed with an intestinalbiopsy. In children, This can beconfirmed by the convincing evidence of serological and genetic evidence.

A strict Gluten-freediet is the main treatment for the Coeliac disease. Oats can be added to the diet as long as there is no provencontamination. Micronutrient Supplementation includes Iron Folic acid VitaminB12 Vitamin D and calcium supplements. If symptoms are persistent another differential diagnosis should beconsidered.

Healthcare professionals Physicians Dieticians andpharmacists should be provided with adequate training to diagnose and supportthe people with coeliac disease. Sufficient information is to be provided topatients and family members on Gluten free diet and supplements, Financial andsocial support available.They should be regularly monitored for adherence and physiologicaland psychological complications.

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