Eosinophilia and EoE

Question 1

Criteria for Hypereosinophilic Syndrome

Answer 1

1. Eos > 1500/ml
2. End organ dysfunction
3. No other etiologies identified

Question 2

EGPA Criteria

Answer 2

ACR Classification requires 4/6
1. asthma
2. sinus abnormality
3. migratory or transient pulmonary opacities
4. mononeuropathy or polyneuropathy
5. >10% of eos on diff
6. biopsy showing eos infiltrate in a blood vessel

Question 3

What is the 5 factor score?

Answer 3

Scoring for EGPA
1. Age >65
2. Myocardial involvement
3. Renal insuff.
4. GI involvement
5. Absence of ENT manifestations (presence is associated with better prognosis)

Question 4

Treatment of EGPA

Answer 4

1. Systemic steroids
2. FFS>/= 2 or FFS of 1 if CV or CNS involvement try cyclophosphamide
3. For maintenance: Aziathioprine, MTX, LEF
4. For refractory cases: Ritux or Mepolizumab

Question 5

Pathophysiology of EoE

Answer 5

• elevated levels of Th2 cytokines IL-4, IL-5, IL-13 and mast cells (which recruit cytokines and eos)

Question 6

Features on endoscopy for EoE

Answer 6

1. Linear furrowing
2. Concentric rings
3. Speckled exudate
4. small calibre esophagus
5. mucosal tears

Question 7

Allergy assessment in EoE. Should it be done?

Answer 7

• SPT and sIgE can be considered
• AAAAI suggests allergy testing based elimination diet over no treatment
• no stat. sig for patch testing
• consider aeroallergen testing
• 10-30% of patients have IgE food allergies
• Use testing to expand a restricted diet

Question 8

Treatment options for EoE

Answer 8

1. Elemental diet, followed by re-intro of 1 food per week starting with less allergenic foods and then the most allergenic foods. alt option: cows milk elim., 6 food elim, 4 food elim
2. Topical CS: Budesonide 1000-2000ug/day, fluticasone prop. 500-1000ug/day
3. Biologics - Dupixent approved FDA 2020
4. Mechanical dilation

Question 9

Categorizing hypereosinophilia

Answer 9

1. Myeloproliferative - PDGFRA1/FGFR/JAK2
2. Lymphoproliferative - CD3+CD4-
3. Famililal
4. Unknown
5. Associated - with IBD or AI conditions

Question 10

List 4 beneficial functions of Eos

Answer 10

1. Helminth defence
2. Adaptive immune system modulation
3. Tissue repair
4. tumour surveillance
5. maintain GI barrier integrity
6. support survival of plasma cells

Question 11

Differences between GERD and EE

Answer 11

GERD
- presents later in life
- more likely to respond to PPI

EE
- associated with food allergies
- has characteristic features on endoscopy like linear furrowing, concentric rings, small calibre esophagus and exudates
- clinical features are dysphagia and food impaction