Good Pasteurs Syndrome - 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

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM) and causes small vessel vasculitis. What is the incidence of GBM?

1 - 1.67 : 1000
2 - 1.67 : 10,000
3 - 1.67 : 100,000
4 - 1.67 : 1,000,000

Answer 4

4 - 1.67 : 1,000,000

Question 5

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM) and causes small vessel vasculitis. Is GBM more common in men or women?

Answer 5

• men
• 2:1

Question 6

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM) and causes small vessel vasculitis. Typically what is the mean age affected (biomodal distribution)?

1 - 30-40 and 70-80
2 - 10-20 and 60-79
3 - 20-30 and 40-50
4 - 20-30 and 60-70

Answer 6

4 - 20-30 and 60-70

Question 7

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM) and causes small vessel vasculitis. What in the basement membrane do the anti-GBM antibodies typically target?

1 - collagen type 1
2 - cartilage
3 - fibroblasts
4 - collagen type IV

Answer 7

4 - collagen type IV

Question 8

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM) and causes small vessel vasculitis. What type of hypersensitivity is GBM?

1 - Type I: IgE mediated antibodies
2 - Type II: cytotoxic reaction mediated by IgG or IgM antibodies.
3 - Type III: immune complex mediated
4 - Type IV: delayed reaction mediated by cellular response

Answer 8

2 - Type II: cytotoxic reaction mediated by IgG or IgM antibodies.

• most commonly IgG that triggers the complement pathway

Question 9

Which 2 tissues are typically affected by Good Pasteurs Syndrome (GBM)?

1 - heart
2 - lungs
3 - kidneys
4 - skin

Answer 9

2 - lungs
- alveolar basement membrane

3 - kidneys
- glomerula basement membrane

Question 10

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM). GBM can then be subdivided into 2 categories based on what?

1 - CRP levels
2 - ESR levels
3 - haematuria
4 - anti-neutrophilic cytoplasmic antibodies (ANCAs)

Answer 10

4 - anti-neutrophilic cytoplasmic antibodies (ANCAs)

• PRS is very dangerous as patients can deteriorate in <48h

Question 11

Good Pasteurs Syndrome (GBM) is an autoimmune condition where antibodies are directed against an antigen intrinsic to the glomerular basement membrane (GBM) and alveolar basement membrane (ABM). Which of the following would NOT be ANCA positive?

1 - Granulomatosis with polyangiitis
2 - Microscopic polyangiitis
3 - Systemic Lupus Erythematous
4 - Eosinophilic granulomatosis with polyangiitis (Churg strauss syndrome)
5 - Drug associated

Answer 11

3 - Systemic Lupus Erythematous

Question 12

Which of the following is NOT typically a risk factor for developing good pastures syndrome (GPS)?

1 - infection
2 - oxidative stress
3 - smoking
4 - female gender
5 - hydrocarbon based solvents

Answer 12

4 - female gender

• HLA-D2 is a genetic risk factor

Question 13

In good pastures syndrome (GPS) the lung symptoms typically present before the kidney symptoms. Which of the following is NOT a common respiratory symptom in GBM?

1 - Shortness of breath
2 - Cough
3 - Haemoptysis
4 - Pulmonary infiltrates on chest radiograph
5 - Decreased carbon monoxide diffusing capacity (DLCO)

Answer 13

5 - Decreased carbon monoxide diffusing capacity (DLCO)

• this is typically increased due to the presence of haemoglobin in the alveoli

GBM eventually leads to a restrictive lung disease

Question 14

In good pastures syndrome (GPS) the kidney and lungs are the most affected organs. Which of the following symptoms is NOT a common kidney symptom in GBM?

1 - proteinuria <3.5g
2 - frothy urine
3 - haematuria
4 - nephritic syndrome

Answer 14

1 - proteinuria <3.5g
- leads to >3.5g proteinuria

Question 15

Which of the following is NOT a common symptom in good pastures syndrome (GPS)?

1 - dysponea/cough
2 - glomerularnephritis
3 - proteinuria >3.5g
4 - haemoptysis

Answer 15

3 - proteinuria >3.5g

Question 16

Typically the gold standard for diagnosing good pastures syndrome (GPS) is a renal biopsy. Which 2 of the following findings would we most likely see?

1 - crescentic glomerulonephritis light microscopy
2 - granulomatosis with eosinophilic infiltration
3 - deposits of IgA
4 - deposits of IgG

Answer 16

1 - crescentic glomerulonephritis light microscopy
4 - deposits of IgG

Question 17

Which of the following antibody can be screened for to diagnose good pastures syndrome (GPS)?

1 - anti-glomerular basement (GBM)
2 - anti-neutrophil cytoplasmic antibodies (ANCA)
3 - cytoplasmic-ANCA
4 - proteinase 3-ANCA

Answer 17

1 - anti-glomerular basement (GBM)
- typically IgG

Question 18

How is good pastures syndrome (GPS) typically treated?

1 - high dose of steroids
2 - cyclophosphamide
3 - plasmapheresis
4 - all of the above

Answer 18

4 - all of the above

• typically does not relapse and is a 1 hit wonder
• plasmapheresis daily 4L exchange for 2 weeks

Question 19

Patients with vasculitis can develop renal manifestations. What % of patients with vasculitis present with renal involvement?

1 - 2%
2 - 20%
3 - 45%
4 - 80%

Answer 19

2 - 20%
- BUT if untreated 80% of patients with vasculitis will develop glomerulonephritis

Question 20

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis. Which of the blood vessels is mainly affected?

1 - large vessels
2 - medium vessels
3 - small vessels
4 - all are affected

Answer 20

3 - small vessels

Question 21

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Is this always symptomatic?

Answer 21

• no
• includes a range from normal eGFR to AKI to rapid progressing glomerulonephritis with need for dialysis
• can also see Pauci-immune crescentic GN

Question 22

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). How can these patients be diagnosed effectively?

1 - presence of anti-neutrophilic cytoplasmic antibody (ANCA)
2 - renal biopsy (Pauci-immune crescentic GN) histology
3 - imaging (CT sinuses, HRCT, CT brain)
4 - bronchoscopy, Lung function tests, nerve conduction studies
5 - all of the above

Answer 22

5 - all of the above

• typically patient presents as someone who just isn’t getting better, but other multi-systemic features that don’t align

Question 23

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Which 2 of the following is important to rule out when thinking of a diagnosis of GN in a patient with vasculitis?

1 - CHF
2 - pulmonary hypertension
3 - TB
4 - infective endocarditis

Answer 23

3 - TB
4 - infective endocarditis

• essentially identify the cause of infection and treat
• then see if symptoms persist

Question 24

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). The 1st line treatment is induction immunosuppression. Which 2 of the following treatment options are used for 3 months to induce remission as part of the induction immunosuppression?

1 - high dose of steroids
2 - cyclophosphamide
3 - azathioprine
4 - mycophenolate mofetil

Answer 24

1 - high dose of steroids
2 - cyclophosphamide

Question 25

20% of patients with vasculitis present with renal manifestations, and if left untreated 80% will develop glomerulonephritis (GN). Following induction immunosuppression with 3 months of high dose steroids and cyclophosphamide. Which 2 of the following treatment options are used in maintenance, due to the high risk of relapse?

1 - methotrexate
2 - opioids
3 - azathioprine
4 - mycophenolate Mofetil

Answer 25

3 - azathioprine
4 - mycophenolate Mofetil

• retuximan should be considered if patient continues to relapse (depletes B and plasma cells)
• plasma exchange can be considered if creatinine is >500

Question 26

What is the mortality rate in 1 year if patients with vasculitis and glomerulonephritis (GN) if left untreated?

1 - 100%
2 - 50%
3 - 25%
4 - 5%

Answer 26

1 - 100%