coeliac disease - sth
TRANSCRIPT
Coeliac Disease:
Symptoms, Diagnosis, Treatment and Management
Dr Matthew Kurien Senior Clinical Lecturer and Honorary Consultant Gastroenterologist, University of Sheffield
Benign Diseases
• 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
Talk Outline
“Some people talk in their sleep. Lecturers talk while other people sleep” - Albert Camus (Nobel Prize for Literature 1957)
4
University of Bologna, c. 1350, Laurentius de Voltolina
Quiz
1. Name these famous people?
2. What do they have
in common?
Gluten Attack!
Damaged Villi Normal Villi
Marsh
Crite
ria
0 Normal
1 Raised Intra epithelial Lymphocytes
(IEL)
2 Raised IEL + Crypt Hyperplasia
3a Partial Villous Atrophy
(PVA)
3b Subtotal villous atrophy
(SVA)
3c Total villous atrophy
TVA)
What is the estimated Prevalence of
Adult Coeliac Disease in the UK?
1:100,000
1:1,000
1:10,000
1:100
Coeliac Disease
The historical prevalence of coeliac disease was 1 in 8000
Coeliac Disease affects ~ 1% of the adult population
• The commonest age for presentation is between the 4th to 6th 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
Coeliac Disease can affect many organs and the gut is just the entrance point
Symptoms of Coeliac Disease
Diarrhoea
Classical Symptoms
Weight Loss Failure to thrive
CD - Symptoms Beyond Diarrhoea, Weight Loss and Anaemia
Diagnosis of Coeliac Disease
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%
Coeliac serology
Schyum et al. UEG Journal 1(5) 319-325
EMA- endomysial Ab; tTG- tissue transglutaminase Ab; DGP - deamidated gliadin peptide
Next steps
• Explain that serology is not diagnostic
• Refer to a GI specialist for biopsy
• Maintain normal diet
NICE Guideline (2015) NG20
• 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??
Consequences of Undiagnosed CD
Ludvigsson et al. UEG Journal 3(2) 106-120
Surgery and CD
Population Screening for CD
Ludvigsson et al. UEG Journal 2015 3(2): 106-120
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.
• 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
Case-finding
NICE Guideline (2015) NG20
• 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
Case-finding
NICE Guideline (2015) NG20
J Pediatr Gastroenterol Nutr. 2012 Jan;54(1):136-60.
To Biopsy or Not Biopsy?
Treatment for CD
Adult adherence to a Gluten-Free Diet
See, Kaukinen, Makharia, Gibson, Murray. Nat. Rev. Gastroenterol. Hepatol. 12, 580–591
CDAT/ Biagi
Assessing Adherence
Adherence
Dietitian Assessment Symptom response Coeliac Serology
Histology
Gluten Peptides
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.
Prescriptions
Helping patients adhere
• Prescriptions been available since 1960
• Not Junk Food – staple foods approved by the Advisory
Committee on Borderline Substances
• Allowances determined by age and sex – Eg. male 19-59 years = 18 units, female 19
- 74 years = 14 units
Bread / rolls
Breakfast cereals
Crackers and crispbreads
Flour / flour-type mixes
Oats
Pasta
Pizza bases
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
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
Follow – up care for CD
CD: The Patient Journey
Primary Secondary
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
NRCD
Review original diagnosis: Biopsy, HLA, serology, FHx
Dietary review
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?
No coeliac disease
Gluten contamination
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
NRCD Algorithm
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. 2013 Jan; 62(1): 43–52.
Grey Cases
NRCD
Refractory Coeliac Disease
Adherence?
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)
Management
• Nutrition, nutrition, nutrition
• Gluten free diet
• Enteral vs PN
• Micronutrients
• Monitor for Re‐feeding Syndrome
Prognosis
• Type1 – 90‐100% 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
Other Gluten related disorders
Allergy
Cianferoni A. Wheat allergy: Diagnosis and Management. journal of Asthma and Allergy 2016:9 13–25
Prevalence: 0.2-1%
Hype?! Health?!
Non Coeliac Gluten (Wheat) Sensitivity
NCGS Prevalence: All self reported
Country Year N % Prevalence
2012 5896 5.9% Secondary care referrals
2012 916 4% NZ children report gluten avoidance
2013 7798 0.6% National Health and Nutrition Examination
Survey
2013 1002 2.9% 13% report symptoms related to gluten population survey but only 3.7% on GFD
2014 910 41.2% Athletes!
2014 1184 9.4% Adult General Population
2015 14701 0.8% National Health and Nutrition Examination
Survey
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
Not just Gluten!!!
The GFD may not be a good thing
Innate Immune
Response?
DEFINE YOUR PATIENT
Mooney PM & Sanders DS Neurogastroenterol Motil 2013
Non
Coeliac
Gluten
Sensitivity
IBS
overlap
Allergy
IgE
Non-IgE
Adaptive Immune
Response
Coeliac
Disease
EMA/TTG
Villous
Atrophy
on Biopsy
FOD
MAP’s
IBS
• 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
Summary
Email: [email protected]
Twitter: @shefgastro. @mattkurien Keep up to date with the latest gastroenterology news in Sheffield through our Twitter feed
Thank you