Eosinophilic granulomatosis with polyangiitis - SMALL CELL

Question 1

In small vessel vasculitis arterioles, capillaries and venules are affected. Which immune cell has been identified as a cause of small cell vasculitis?

1 - B cells
2 - T cells
3 - NK cells
4 - neutrophils

Answer 1

1 - B cells
- specifically targets granuloes (neutrophils, basinophils and eosinophils)

Question 2

In small vessel vasculitis arterioles, capillaries and venules are affected. B cells produce antibodies against granuoles (neutrophils, basophils and eosinophils). What are these antibodies called?

1 - granuoles antibodies
2 - rheumatoid factor
3 - anti-neutrophilic cytoplasmic antibodies (ANCAs)
4 - MHC-I antibodies

Answer 2

3 - anti-neutrophilic cytoplasmic antibodies (ANCAs)

Question 3

In small vessel vasculitis arterioles, capillaries and venules are affected. B cells produce antibodies against granuoles called anti-neutrophilic cytoplasmic antibodies (ANCAs). What is the most common form of antibody produced?

1 - IgA
2 - IgG
3 - IgM
4 - IgD

Answer 3

2 - IgG

Question 4

Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis. What is the incidence of EGPA?

1 - 0.2 : 100,000
2 - 20 : 100,000
3 - 200 : 100,000
4 - 2000 : 100,000

Polyangiitis
- poly = many
- angiitis = vasculitis

Granulomatosis
- granuloma = immune cell warded off area

Answer 4

1 - 0.2 : 100,000
- highest prevalence is in Australia

Question 5

Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis. What is the peak age that this occurs in?

1 - 20-30
2 - 30-40
3 - 40-50
4 - 60-60

Polyangiitis
- poly = many
- angiitis = vasculitis

Granulomatosis
- granuloma = immune cell warded off area

Answer 5

3 - 40-50

Question 6

Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis. Does EGPA affect men or women more, or is it equal?

Polyangiitis
- poly = many
- angiitis = vasculitis

Granulomatosis
- granuloma = immune cell warded off area

Answer 6

• affects both equally

Question 7

Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis. Which of the following is NOT one of the classical triad of clinical features of EGPA?

1 - conjunctivitis
2 - rhinitis
3 - peripheral eosinophilia
4 - asthma

Answer 7

1 - conjunctivitis

Asthma is most common feature
- occurs in 97% of patients with EGPA

Question 8

Which of the following are typical constitutional symptoms in patients with Eosinophilic granulomatosis with polyangiitis (EGPA)?

1 - malaise
2 - fatigue
3 - flulike symptoms
4 - weight loss
5 - fever
6 - myalgias (aches and pains)
7 - all of the above

Answer 8

7 - all of the above

Question 9

All of the following tissues can be affected in Eosinophilic granulomatosis with polyangiitis (EGPA), but which is most common?

1 - Skin
2 - Cardiac
3 - GI
4 - Lungs

Answer 9

4 - Lungs
- skin is 2nd most affected

Question 10

When trying to diagnose patients with Eosinophilic granulomatosis with polyangiitis (EGPA), which immune can be significantly raised in serum?

1 - basinophils
2 - eosinophils
3 - T helper cells (CD4)
4 - macrophages

Answer 10

2 - eosinophils
- called eosinophilia
- >10% increase or absolute count 4000-9000) and anaemia

Question 11

When trying to diagnose patients with Eosinophilic granulomatosis with polyangiitis (EGPA), 2 inflammatory markers are typically raised. Which 2 of the following?

1 - C3 (complement pathway)
2 - ESR
3 - CRP
4 - serum amyloid A

Answer 11

2 - ESR
3 - CRP

Question 12

When trying to diagnose patients with Eosinophilic granulomatosis with polyangiitis (EGPA), we can search for specific antibodies. EGPA results in a specific type of auto antibody to be released called perinuclear anti-neutrophilic cytoplasmic antibodies (pANCAs). pANCAs bind with a specific antigen on granules called what?

1 - myeloperoxidase
2 - elastin
3 - proteinase-3
4 - lipoprotein-3

Answer 12

1 - myeloperoxidase
- gives MPO-EGPA
- positive in 40% of patients

Question 13

Lungs are the most common tissue affected in Eosinophilic granulomatosis with polyangiitis (EGPA), what might we see in a chest X-ray or CT scan?

1 - malignancy
2 - nodular pulmonary opacities
3 - emphysema
4 - fibrosis

Answer 13

2 - nodular pulmonary opacities

Question 14

If a biopsy was taken in patients with Eosinophilic granulomatosis with polyangiitis (EGPA), which of the following would we NOT see?

1 - Eosinophilic infiltration
2 - extensive areas of necrosis
3 - eosinophilic, giant cell vasculitis, especially of the small arteries and veins
4 - interstitial and perivascular necrosis but no granulomas

Answer 14

4 - interstitial and perivascular necrosis but no granulomas

• We would see Interstitial and perivascular necrotizing granulomas

Question 15

Which of the following is NOT a treatment used to induce induction in Eosinophilic granulomatosis with polyangiitis (EGPA) when there is organ- or life-threatening implications?

1 - Glucocorticoids in combination with eitherrituximaborcyclophosphamide
2 - Plasma exchange
3 - Methotrexate
4 - Avacopan—C5a receptor inhibitor

Answer 15

3 - Methotrexate

• relapse is common because cANCA remains

Question 16

Which of the following is NOT a treatment used in maintenance therapy in Eosinophilic granulomatosis with polyangiitis (EGPA)?

1 - Azathioprine
2 - Mycophenolate mofetil (MMF)
3 - Methotrexate
4 - Avacopan—C5a receptor inhibitor

Answer 16

4 - Avacopan—C5a receptor inhibitor