22. Vasulitis

Question 1

What is vasculitis?

Answer 1

Inflammation within blood vessels which distrupts the internal elastic lamina

Question 2

What kind of necrosis can be caused by vasculitis?

Answer 2

Fibrinoid

Question 3

What are the features of widespread inflammation?

Answer 3

Temperature
Malaise
Weight loss
Fatigued

Question 4

What are the causes of secondary systemic vasculitis?

Answer 4

Infection, drugs, malignancy
Cryoglobulinaemia
Connective tissue disease

Question 5

It is common for vessels to rupture from vasculitis. T/F?

Answer 5

False

Question 6

How is primary vasculitis classified?

Answer 6

Size of vessel

Whether or not there is granuloma formation

Question 7

What type of primary granuloma occurs within large vessels?

Answer 7

Temporal arteritis

Question 8

What type of primary granuloma occurs within medium vessels and is associated with granulomata?

Answer 8

EGPA

Eosinophilic Granulomatosis with Polyangitis

Question 9

Which types of primary granuloma occur within medium vessels and are not associated with granulomata?

Answer 9

Polyarteritis nodosa

Kawasaki syndrome

Question 10

What type of primary granuloma occurs in small vessels and is associated with granulomata formation?

Answer 10

GPA

Granulomatosis with Polyangiitis

Question 11

What type of primary granuloma occurs in small vessels and is not associated with granulomata formation?

Answer 11

Microscopic polyangitis

Henoch Schonlein purpura

Question 12

What are the causes of mortality in vasculitis?

Answer 12

Due to renal, pulmonary and neuro disease

Effects of immunosuppressive treatment

Question 13

How is vasculitis diagnosed?

Answer 13

ANCA on serology
Biopsy of affected organ
Angiography in large vessel disease to show aneurysm formation

Question 14

What conditions cause a false positive ANCA?

Answer 14

Infection
Inflammatory disease
Lymphoma

Question 15

What organs are most common affected by GPA?

Answer 15

Respiratory tract
-nose bleeds
Glomerulonephritis

Question 16

What is ANCA?

Answer 16

Collection of autoantibodies against neutrophils

Question 17

What are the types of ANCA and what do they bind to?

Answer 17

cANCA binds to PR3

pANCA binds to MPO proteins in the cytoplasm of the neutrophil

Question 18

Which type of ANCA is more closely associated with vasculitis?

Answer 18

cANCA

Question 19

What types of infection can cause secondary vasculitis?

Answer 19

Subacute Bacterial Endocarditis

Cystic Fibrosis

Question 20

What is the difference between acute and subacute bacterial endocarditis?

Answer 20

Acute directly due to infection

Subacute presentation is dominated by immune complex destruction

Question 21

What are the features of subacute bacterial endocarditis?

Answer 21

Valve damage
Immune complex deposition
Widespread inflammation

Question 22

How is subacute bacterial endocarditis diagnosed?

Answer 22

Pyrexia of unknown origin
3 blood cultures
Vegetations on echo
Acute phase response

Question 23

What are cryoglobulins?

Answer 23

Igs which stick together in the cold

Question 24

What causes the features of cryoglobulinaemia?

Answer 24

Vascular spasm

Vasculitis

Question 25

What is the dermatological presentation of cryoglobulinaemia?

Answer 25

Palpable purpura | -Bumpy due to damage to the blood vessel

Question 26

What is Raynaud's phenomenon?

Answer 26

When exposed to the cold, vascular spasm causes fingers to turn white and blue

Question 27

How does cryoglobulinaemia present?

Answer 27

Skin and Raynaud's Joint, renal, neuro and GI involvement Vasculitis

Question 28

What is Meltzer's triad of cryglobulinaemia?

Answer 28

Purpura Arthralgia Weakness

Question 29

How is serum tested for cryoglobulinaemia?

Answer 29

Must be kept at 37C until it is separated in the lab | Kept at 4C and read 3 days later

Question 30

What causes type one cyroglobulinaemia?

Answer 30

Monoclonal | Myeloma, Waldenstrom's, B cell lymphoma

Question 31

What causes type two cryoglobulinaemia?

Answer 31

Mixed monoclonal and polyclonal | Infection, connective tissue disease, lymphoproliferative disease

Question 32

What causes type three cryoglobulinaemia?

Answer 32

Polyclonal | Connective tissue disease, chronic infection

Question 33

What is the treatment for cryoglobulinaemia?

Answer 33

Treat the underlying cause Steroids/ immunosuppression for vasculitis Plasmaphoresis if life threatening Rituximab to deplete B cells