Coeliac Disease: Symptoms, Diagnosis, Treatment and Management
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1 Coeliac Disease: Symptoms, Diagnosis, Treatment and Management Dr Matthew Kurien Senior Clinical Lecturer and Honorary Consultant Gastroenterologist, University of Sheffield
2 Benign Diseases
3 Talk Outline Review the CD Basics Hot topics Diagnosis Detection/ Serology/ Paeds vs Adults Treatment Gluten Free Diet/ Adherence/ Prescriptions Follow-up Problems/ Non-responders/ Refractory CD Other Gluten related disorders
4 Some people talk in their sleep. Lecturers talk while other people sleep - Albert Camus (Nobel Prize for Literature 1957) University of Bologna, c. 1350, Laurentius de Voltolina 4
5 Quiz 1. Name these famous people? 2. What do they have in common?
6
7 Gluten Attack! Normal Villi Damaged Villi
8 0 Normal 1 Raised Intra epithelial Lymphocytes (IEL) Marsh Criteria 2 Raised IEL + Crypt Hyperplasia 3a Partial Villous Atrophy (PVA) 3b Subtotal villous atrophy (SVA) 3c Total villous atrophy TVA)
9 Coeliac Disease What is the estimated Prevalence of Adult Coeliac Disease in the UK? 1:100,000 1:1,000 1:10,000 1:100
10 The historical prevalence of coeliac disease was 1 in 8000
11 Coeliac Disease affects ~ 1% of the adult population The commonest age for presentation is between the 4 th to 6 th Decade For every paediatric case diagnosed there are 9 adult cases Coeliac disease affects 1% of the adult population! For every adult case diagnosed there are 3-4 cases still not recognised People with undiagnosed adult coeliac disease generally have a normal BMI and may even be overweight
12 Coeliac Disease can affect many organs and the gut is just the entrance point
13 Symptoms of Coeliac Disease
14 Classical Symptoms Diarrhoea Weight Loss Failure to thrive
15 CD - Symptoms Beyond Diarrhoea, Weight Loss and Anaemia
16 Diagnosis of Coeliac Disease
17 Diagnosis - Tip of the Iceberg 1 in every 4 CD patients detected Delays Norstrom et al. BMC Gastro 2011 Symptoms to Diagnosis 9.7 years 1st Doctor to Diagnosis 5.8 years 5%
18 Coeliac serology EMA- endomysial Ab; ttg- tissue transglutaminase Ab; DGP - deamidated gliadin peptide Schyum et al. UEG Journal 1(5)
19 Next steps Explain that serology is not diagnostic Refer to a GI specialist for biopsy Maintain normal diet NICE Guideline (2015) NG20
20 Consequences of Undiagnosed CD Symptomatic Diminished QoL Increased presentations to healthcare professionals Increased medical interventions (abdominal surgery/ endoscopy) Adverse pregnancy outcomes Increased lymphoma Mortality? Asymptomatic Uncertainty if reduces risk of complication Improves QOL?? Ludvigsson et al. UEG Journal 3(2)
21 Surgery and CD
22 Population Screening for CD Ludvigsson et al. UEG Journal (2):
23 Case-finding for CD Case-finding is a strategy for targeting resources at individuals or groups who are suspected to be at risk for a particular disease.
24 Case-finding Offer serological testing to: People with any of the following: persistent unexplained abdominal or gastrointestinal symptoms faltering growth prolonged fatigue unexpected weight loss severe or persistent mouth ulcers unexplained iron, vitamin B12 or folate deficiency type 1 diabetes, at diagnosis autoimmune thyroid disease, at diagnosis irritable bowel syndrome (in adults) first-degree relatives of people with coeliac disease NICE Guideline (2015) NG20
25 Case-finding Consider serological testing metabolic bone disorder (reduced bone mineral density or osteomalacia) unexplained neurological symptoms (particularly peripheral neuropathy or ataxia) unexplained subfertility or recurrent miscarriage persistently raised liver enzymes with unknown cause dental enamel defects Down's syndrome Turner syndrome NICE Guideline (2015) NG20
26 To Biopsy or Not Biopsy? J Pediatr Gastroenterol Nutr Jan;54(1):
27 Treatment for CD
28 Adult adherence to a Gluten-Free Diet See, Kaukinen, Makharia, Gibson, Murray. Nat. Rev. Gastroenterol. Hepatol. 12,
29 Assessing Adherence Dietitian Assessment Symptom response Coeliac Serology CDAT/ Biagi Adherence Gluten Peptides Histology
30 What factors influence Adherence? Cognitive, emotional and socio-cultural influences Membership of an advocacy group eg. Coeliac UK Regular dietetic follow-up Prescriptions for Gluten Free foods Hall NJ et al. Intentional and inadvertent non-adherence in adult coeliac disease. A cross-sectional survey. Appetite 2013;68:56-62.
31 Prescriptions
32 Helping patients adhere Prescriptions been available since 1960 Not Junk Food staple foods approved by the Advisory Committee on Borderline Substances Bread / rolls Breakfast cereals Crackers and crispbreads Flour / flour-type mixes Oats Pasta Pizza bases Allowances determined by age and sex Eg. male years = 18 units, female years = 14 units
33 Fact or Fiction? No budget supermarkets had GF foods Large supermarkets and online- median 22 items GF food 3-4 x more expensive than non GF foods
34 GF Prescriptions Cost 25.7 million (0.3% of prescribing budget) 40% of CCGs is England- restricting or removing these prescriptions for CD and DH patients
35 Follow up care for CD
36 CD: The Patient Journey
37 Non Responsive Coeliac Disease (NRCD) Definition persistent symptoms, signs or laboratory abnormalities typical of CD despite 6 12 months of dietary gluten avoidance 7-30% of all CD patients Primary Secondary
38 NRCD Algorithm NRCD Review original diagnosis: Biopsy, HLA, serology, FHx No coeliac disease Dietary review Gluten contamination Repeat gastroscopy with biopsy and aspirate Colonoscopy and biopsy Faecal elastase Stool culture Bloods inc inflammatory markers, thyroid function Exclude other causes: SBBO PEL Fructose intolerance Lactose intolerance Consider RCD? Microscopic colitis Exocrine pancreatic insufficiency Giardiasis Hyperthyroidism Figure 1. Diagnostic algorithm for NRCD. FHx= Family History NRCD= Non-responsive coeliac disease; PLE= Protein losing enteropathy; SBBO=small bowel bacterial overgrowth; RCD= Refractory coeliac disease. BSG guidelines adopted from Mooney et al. JGLD 2012
39 Refractory Coeliac Disease Definition Persistent or recurrent malabsorptive symptoms and signs with villous atrophy, despite a strict gluten free diet for more than 12 months. The Oslo definitions for coeliac disease and related terms. Ludvigsson et al. Gut Jan; 62(1): NRCD Adherence? Grey Cases Refractory Coeliac Disease
40 Refractory coeliac disease Rare cause of NRCD unknown true incidence? 1.5% Diagnosis of exclusion Persistent changes of CD despite strict adherence to GFD Pre malignant condition Type 1 polyclonal expansion of IELs and villous atrophy Type 2 includes ulcerative jejunitis, clonal expansion of abberant IELs (CD8+ TCR γδ cells) Enteropathy Associated T cell Lymphoma (EATL)
41 Management Nutrition, nutrition, nutrition Gluten free diet Enteral vs PN Micronutrients Monitor for Re feeding Syndrome
42 Prognosis Type % 5 year survival Type2 50% 5 year survival Ulcerative jejunitis Dismal EATL 20 30% 2 year survival 50 times more common in someone with coeliac disease, the annual incidence is low (0.5 1 per million people) Not necessarily a linear progression
43 Other Gluten related disorders
44 Allergy Prevalence: 0.2-1% Cianferoni A. Wheat allergy: Diagnosis and Management. journal of Asthma and Allergy 2016:
45 Non Coeliac Gluten (Wheat) Sensitivity Hype?! Health?!
46 NCGS Prevalence: All self reported Country Year N % Prevalence % Secondary care referrals % NZ children report gluten avoidance % National Health and Nutrition Examination Survey % 13% report symptoms related to gluten population survey but only 3.7% on GFD % Athletes! % Adult General Population % National Health and Nutrition Examination Survey
47 Gluten-free diets gaining in popularity: Health or hype: USA today 2008 Reuter s online GFD $1.31 billion in US 2011 $ 1.68 billion in 2015
48 Not just Gluten!!!
49 The GFD may not be a good thing
50 DEFINE YOUR PATIENT Mooney PM & Sanders DS Neurogastroenterol Motil 2013 Coeliac Disease EMA/TTG Villous Atrophy on Biopsy Allergy IgE Non-IgE Non Coeliac Gluten Sensitivity IBS overlap FOD MAP s IBS Adaptive Immune Response Innate Immune Response?
51 Summary Review the CD Basics Hot topics Diagnosis Detection/ Serology/ Bulb biopsies/ Paeds vs Adults Treatment Gluten Free Diet/ Adherence/ Prescriptions Follow-up Problems/ Non-responders/ Refractory CD Other Gluten related disorders
52 Thank you Keep up to date with the latest gastroenterology news in Sheffield through our Twitter feed
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