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Coeliac Disease - Coggle Diagram
Coeliac Disease
Suspicion
Persistent anemia
Iron, Folate- and/or Vitamin B12-deficiency
Positive Serology
Fatigue
Wheight loss
Elevated liver encymes of unkown cause (associated with autoimmune hepatitis)
Subfertility/Misscarriage
Therapy
Life-long gluten-free diet (counsel for strict adherence incl. eating out and not sharing plates with family who do not eat gluten-free)
Dietist
Treatment goal: Resolution of symptoms, mucosal healing
Disease advocacy group
Recommend the use of gluten-free oats (improved cardiovascular profile)
Low adherence is associated with lymphoproliferative malignancy (but only if duodenal changes persist)
Diagnosis
Continue gluten-containing (3-6g gluten daily) diet before diagnosis (min. 6 weeks)
Ig-Serology
IgA Tissue Transglutaminase
Total IgA
IgG coeliac serology if IgA negative/deficiency
Deamidated gliadin peptides-IgA
Upper endoscopy
4 biopsies from Duodenum Pars 2
2 biopsies from Duodenum Pars 1 (Bulbus)
Biopsies should be single bites
Histology
Use histological grading system
Marsh-Garding
Marsh 1: > 25 IEL/100 Epithelial Cells, normal crypts, normal vili
Marsh 2: > 25 IEL/100 Epithelial Cells, hyperplastic crypts, normal vili
Marsh 3: > 25 IEL/100 Epithelial Cells, hyperplastic crypts, Villous atrophy, 3 sublevels (moderate or strong atrophy, missing; unknown clinical relevance)
Marsh 0: Normal
HLA-Testing
DQ2 and DQ8 can be tested to exclude coeliac disease
Diagnostic criteria
Definitive
Positive coeliac serology
Increased intraepithelial lymphocytes (> 25/100 epithelial cells) + crypt hyperplasia with/without villous atrophy
Potential
Persistently positive serology
Isolated increased intraepithelial lymphocytes (IEL, > 25 IEL/100 epithelial cells)
HLA-DQ2/HLA-DQ8 phenotype
Seronegative
Villous atrophy (Marsh 3)
HLA-DQ2/HLA-DQ8 phenotype
improvement on gluten-free diet
Serology-only
IgA-tTG titre >= 10 x Upper Limit
Only shared decision making with patient
Follow-up
Regular for 2 years after diagnosis
Longer if no adherence to diet, no response to diet or complications
Repeat biopsy and serology if gluten-diet continued and CD probable
Only biopsy can assess healing
Blood: Blood count, liver enzymes, Iron, Folate, Vitamin B12, Vitamin D, Calcium (Deficiencies may arise due to lack of micronutrients in gluten-free diet!)
DXA scan 1 year after beginning diet (and every 2-3 years if persistent villous atrophy)
Vaccination
Pneumococci
Serology: Ig decrease within weeks/months on gluten-free diet (recurrence only appears after > 4 weeks exposure, so intermittent exposure cannot be assessed) --> rather insufficient marker, once gluten-free diet is started
Refractory coeliac disease (RCD)
See Algorithm in Fig. 3 (BSG Coeliac Disease, p. 20)
= Persistence/Reemergence of symptoms/signs/lab abnormalities with ongoing villous atrophy despite 12 months of gluten-free diet
Most common causes: Exposition to gluten, IBS/functional, IBD, microscopic colitis, slow healing
Typically in patients with diagnosis at age > 50 years
RCD Type 1
Diagnosis of exclusion
Histology: Villous atrophy, IEL similar to active CD
Benign course (5-year-survival 80-95%)
Treatment: Oral immunosuppression (open-capsule budesonide or thiopurines for steroid-sparing agents for long-term treatment)
Follow-up 12-monthly
RCD Type 2
Low-grade intraepithelial cell lymphoma
Aggressive course (5-year-survival 50%, 10% if T-cell lymphoma)
Histology: Villous atrophy, IEL similar to active CD
Immunohistology: Aberrant IEL = expression of intracellular CD3, loss of surface CD8 (>= 40% of IEL)
Association: Bleeding, Fever, Night sweats, Ulcerative Jejunitis, enteropathy-associated T-cell lymphoma
Flow cytometry: Assess for CD45, CD7, CD103, surface CD3, cytoplasmatic CD3 ( >= 20% sCD3-/cCD3+ lymphocytes of total population is diagnostic)
Therapy: Cladribin (Adenosin-Nucleotide-Analogon)
Follow-up 6-monthly
Screening, when (and for)...
IBD, Irritable Bowel Syndrome, Microscopic Colitis, Autoimmune Atrophic Gastritis
Diabetes mellitus Type 1, Autoimmune Thyreoiditis, Addison
Autoimmune Hepatitis, Hyposplenism, Idiopathic Pancreatitis
First-degree relatives with CD
Dermatitis herpetiformis, Sjögren Syndrome
Differentialdiagnosis
Serology
False-positive
Chronic liver disease, autoimmune diseases, enteric infections
False-negative
Immunsuppression, Immunodeficiency, Gluten withdrawal
Histology
False-positive
Interobserver variability, sampling artefact
False-negative
Interobserver variability, gluten withdrawal, sampling error
For increased IEL and/or villous atrophy
Infections
H. pylori, Viral gastroenteritis, Giardiasis, Cryptosporidium, Whipple Disease, AIDS Enteropathy
Drugs
NSAID, PPI, Mycophenolat, Sartan, Methotrexat, Immun-Checkpointinhibitors, Radiochemo
Other
M. Crohn, Microscopic Colitis, CVID, GvH-Disease, Autoimmune Enteropathy, SIBO, Peptic Duodenitis, Eosinophilic Enteritis, Amyloidosis