Vasculitis

Question 1

Vasculitis definition

Answer 1

Autoimmune disease causing blood vessel inflammation

Question 2

Vasculitis can lead to… (3) (types of damage)

Answer 2

• stenosis (reduced lumen size)
• occlusion
• aneurysms (outpouching)

Question 3

2 types of vasculitis

Answer 3

1. Primary (occurring by itself)
2. Secondary (occurring in association with another disease like lupus)

Question 4

3 types of primary vasculitis (hint: depends on vessel size)

Answer 4

1. Large vessel vasculitis
2. Medium vessel vasculitis
3. Small vessel vasculitis

Question 5

The type of vasculitis is determined based on affected organs.
If kidneys and lungs are affected, what type of vasculitis do we suspect?

Answer 5

Small vessel vasculitis

Question 6

The type of vasculitis is determined based on affected organs.
If skin, peripheral nerves and GI tract are affected, what type of vasculitis do we suspect?

Answer 6

Small and medium vessel vasculitis

Question 7

The type of vasculitis is determined based on affected organs.
If the aorta and its primary branches are affected, what type of vasculitis do we suspect?

Answer 7

Large vessel vasculitis

Question 8

Small vessel vasculitis: 3 physical exam findings

Answer 8

1. Splinter hemorrhages
2. Palpable purpura
3. Digital ischemia (finger necrosis)

Question 9

Medium vessel vasculitis: 2 physical exam findings

Answer 9

1. Livedo reticularis (net-like red-blue discoloration)
2. Vasculitis nodules on hands

Question 10

What is a symptom of neurological involvement as a result of small/medium vessel vasculitis?

Answer 10

Mononeuritis Multiplex (damage to peripheral nerves by immune system and inflammation)

Question 11

Physical exam findings resulting from peripheral nerve damage secondary to vasculitis? (2)

Answer 11

Dropped hand or dropped foot and other abnormalities on peripheral neurological exam.

Question 12

What type of autoantibody is strongly associated with small vessel vasculitis?

Answer 12

Anti-Neutrophil Cytoplasmic Antibodies (ANCA)

Question 13

2 types of ANCAs

Answer 13

cANCA: cytoplasmic
pANCA: perinuclear

Question 14

cANCAs and pANCAs are directed against 2 different proteins…

Answer 14

cANCA: neutrophil proteinase 2 (PR3)
pANCA: neutrophil myeloperoxidase (MPO)

Question 15

cANCA is associated with a specific type of vasculitis called…

Answer 15

granulomatosis with polyangiitis (GPA)

Question 16

pANCA is associated with 2 specific types of vasculitis called…

Answer 16

• microscopic polyangiitis (MPA)
• eosinophilic granulomatosis with polyangiitis

Question 17

3 types of ANCA-associated vasculitis

Answer 17

1. Granulomatosis with polyangiitis
2. Eosinophilic granulomatosis with polyangiitis
3. Microscopic polyangiitis

Question 18

Peak age of onset for ANCA-associated vasculitis

Answer 18

45-74

Question 19

ANCA-associated vasculitis is more common in…
a) females
b) males

Answer 19

b) males (1.3:1)

Question 20

Describe granulomatosis with polyangiitis (GPA)

Answer 20

• Necrotizing granulomatous inflammation of the upper and lower respiratory tract (sinuses, nose, trachea, bronchi, lungs)
• Necrotizing vasculitis of the small arteries and veins

Question 21

4 clinical manifestations of granulomatosis with polyangiitis (GPA)

Answer 21

1. Upper or lower respiratory tract symptoms (see slides)
2. Necrotizing pauci-immune glomerulonephritis (kidneys)
3. Arthritis
4. Purpura

Question 22

Physical signs of damage caused by granulomatosis with polyangiitis (3)

Answer 22

1. Saddle-nose deformity (due to cartilage destruction)
2. Perforated septum
3. Eye involvement and inflammation

Question 23

Describe microscopic polyangiitis (MPA)

Answer 23

• Similar to GPA but occurs in older individuals
• Upper respiratory tract involvement is much less frequent
• No immune complex deposition (pauci-immune)
• Necrotizing vasculitis of small vessels

Question 24

3 clinical manifestations (cardinal features) of microscopic polyangiitis (MPA)

Answer 24

1. Glomerulonephritis
2. Pulmonary hemorrhage
3. Mononeuritis multiplex (i.e. multiple mononeuropathies)

Question 25

How do we diagnosis microscopic polyangiitis (MPA)?

Answer 25

* pANCA present in 50-80% of patients * biopsy should be obtained

Question 26

Describe eosinophilic granulomatosis with polyangiitis (EGPA)

Answer 26

Eosinophil-rich and necrotizing granulomatous inflammation often involving respiratory tract, with necrotizing small vessel vasculitis

Question 27

3 key features (triad) of eosinophilic granulomatosis with polyangiitis (EGPA)

Answer 27

- Asthma - Eosinophilia - Systemic vasculitis

Question 28

EGPA: Progression of disease (3 phases)

Answer 28

1. Allergic rhinitis and asthma (often steroid-dependent and becomes increasingly severe) 2. Peripheral eosinophilia and eosinophilic infiltrates in many organs 3. Vasculitic disease involving nerves, lungs, heart, GI tract, kidneys

Question 29

What antibody is associated with GPA?

Answer 29

cANCA (anti-PR3)

Question 30

What antibody is associated with MPA?

Answer 30

pANCA (anti-MPO)

Question 31

What antibody is associated with EGPA?

Answer 31

pANCA (anti-MPO)

Question 32

Review Key Clinical Pearls! Last slide

Answer 32

:)