Granulomatosis with polyangiitis (GPA) - 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

What is the incidence of granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA)?

1 - 0.1 cases per 100,000
2 - 1 cases per 100,000
3 - 10 cases per 100,000
4 - 100 cases per 100,000

Answer 4

2 - 1 cases per 100,000

Question 5

Is granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA) more common in men or women?

Answer 5

• men
  1.5 : 1

Question 6

What is the peak age for the incidence of granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA)?

1 - 30-40
2 - 40-50
3 - 50-70
4 - 60-70

Answer 6

4 - 60-70

Question 7

Is granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA) more common in Europeans of asians?

Answer 7

• europeans
• rare in asians

Question 8

Granulomatosis with polyangiitis (inflammation of multiple blood vessels) (GPA), previously referred to as Wegners Granulomatosis, is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called what?

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

Answer 8

1 - cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs)

Small proportion of patients can be positive for perinuclear ANCA (pANCA)

Question 9

Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). cANCAs bind with a specific antigen on granules called what?

1 - MHC-I
2 - elastin
3 - proteinase-3
4 - lipoprotein-3

Answer 9

3 - proteinase-3 (PR3)
- PR3-cANCA

• cANCA binding to proteinase-3 causes cytokines to be released onto endothelium causing vasculitis

Question 10

Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which of the following small blood vessels is tissues is NOT typically affected by GPA?

1 - nasopharynx (ear, nose and throat (ENT))
2 - heart and aorta
3 - airways and lungs
4 - kidneys
5 - skin
6 - eyes and PNS

Answer 10

2 - heart and aorta

• pauci-immune necrotizing glomerulonephritis is common in kidneys
• necrotizing granulomatous inflammation affecting small-to-medium vessels

Question 11

What markers of renal function in Granulomatosis with polyangiitis (GPA) are abnormal if the kidney is affected?

1 - proteinuria
2 - haematuria and red cell casts
3 - elevated creatinine
4 - urinary sediment
5 - all of the above

Answer 11

5 - all of the above

Question 12

Which 2 of the following inflammatory markers are commonly raised in Granulomatosis with polyangiitis (GPA)?

1 - ESR
2 - CRP
3 - IL-6
4 - TNF-a

Answer 12

1 - ESR
2 - CRP

Question 13

Granulomatosis with polyangiitis (GPA), previously referred to as Wegners Granulomatosis is a small vessel vasculitis. GPA results in a specific type of auto antibody to be released called cytoplasmic anti-neutrophilic cytoplasmic antibodies (cANCAs). Which of the following is NOT a symptom patients typically present with?

1 - blood mucus from nose
2 - pain from sinusitis
3 - saddle nose
4 - SOB with blood on cough
5 - increased BP and urine output

Answer 13

5 - increased BP and urine output
- causes increased BP and and reduced urine output

• Saddle nose caused by bone and cartilage destruction
• SOB caused by interstitial lung disease, which can be detected in X-rays or CT scans, may also identify nasopharynx impairments

Question 14

Which of the following is NOT a treatment used to induce induction in granulomatosis with polyangiitis (GPA) 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 14

3 - Methotrexate

• relapse is common because cANCA remains

Question 15

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

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

Answer 15

4 - Avacopan—C5a receptor inhibitor