8.2. Mulit-System Autoimmune Disease - Systemic Vasculitis Diseases

Question 1

What are the 3 types of Blood Vessels, affected by Systemic Vasculitis Diseases?

Answer 1

1. Large Vessel Vasculitis
2. Medium Vessel Vasculitis
3. Small Vessel Vasculitis

Question 2

What is the common Large Vessel Vascuilitis Condition?

Answer 2

Giant Cell Arteritis

Question 3

What are the common Medium Vessel Vascuilitis Conditions?

Answer 3

1, Polyarteritis Nodosa

2. Kawasaki Disease

Question 4

What are the common Small Vessel Vascuilitis Conditions?

Answer 4

These are known as ANCA-Associated Vasculitis and include:

1. Granulomatosis Polyangiitis (Wegner’s Granulomatosis)
2. Microscopic Polyangiitis
3. Eosinophilic Granulomatosis Polyangiitis

Question 5

What is Giant Cell Arteritis?

Answer 5

Inflammation of the Arterial Wall with Fragmentation and Disruption of the Internal Elastic Lamina

Question 6

What is the Diagnostic Criteria for Giant Cell Arteritis?

Answer 6

3 of the following:

1. Age at onset ? 50 years
2. New Headache
3. Temporal Artery Tenderness / Reduced Pulsation
4. ESR > 50
5. Abnormal Temporal Biopsy

Question 7

What are the 3 ANCA-associated Vasculitis Conditions?

Answer 7

1. Wegner’s Granulomatosis - Granulomatosis with Polyangiitis
2. Microscopic Polyangiitis
3. Eosinophilic Granulomatosis with Polyangiitis

Question 8

What is the pathophysiology of Wegner’s Granulomatosis (Granulomatosis with Polyangiitis)?

Answer 8

Necrotising Granulomatous Inflammation (involving the Respiratory Tract)

Question 9

What size vessels does Wegner’s Granulomatosis (Granulomatosis with Polyangiitis) affect?

Answer 9

Small - Medium Vessels

Question 10

What is commonly associated with Wegner’s Granulomatosis (Granulomatosis with Polyangiitis)?

Answer 10

Necrotising Glomerulonephritis

Question 11

What is the pathology of Microscopic Polyangiitis?

Answer 11

1. Necrotising Vasculitis (with few/no immune deposits)

2. Necrotising Arteritis

Question 12

What size Vessels does Microscopic Polyangiitis commonly affect?

Answer 12

Small - Medium

Note - Mainly small

Question 13

What is commonly associated with Microscopic Polyangiitis?

Answer 13

1. Necrotising Glomerulonephritis

2. Pulmonary Capillaritis

Question 14

Is Granulomatous Inflammation present in Microscopic Polyangiitis?

Answer 14

No - Few / No immune deposits

Question 15

What is the pathophysiology of Eosinophilic Granulomatosis with Polyangiitis?

Answer 15

Eosinophil Rich and Necrotising Granulomatous Inflammation (involving the Respiratory Tract)

Question 16

What size Vessels does the Necrotising Vasculitis, associated with Eosinophilic Granulomatosis with Polyangiitis, affect?

Answer 16

Small - Medium Vessels

Question 17

What is commonly associated with Eosinophilic Granulomatosis with Polyangiitis?

Answer 17

1. Asthma

2. Eosinophilia

Question 18

What is the relationship between ANCA and Glomerulonephritis?

Answer 18

ANCA is more frequent when Glomerulonephritis is present