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BLOCK F > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Vasculitis definition

A

Autoimmune disease causing blood vessel inflammation

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2
Q

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

A
  • stenosis (reduced lumen size)
  • occlusion
  • aneurysms (outpouching)
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3
Q

2 types of vasculitis

A
  1. Primary (occurring by itself)
  2. Secondary (occurring in association with another disease like lupus)
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4
Q

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

A
  1. Large vessel vasculitis
  2. Medium vessel vasculitis
  3. Small vessel vasculitis
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5
Q

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

A

Small vessel vasculitis

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6
Q

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?

A

Small and medium vessel vasculitis

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7
Q

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?

A

Large vessel vasculitis

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8
Q

Small vessel vasculitis: 3 physical exam findings

A
  1. Splinter hemorrhages
  2. Palpable purpura
  3. Digital ischemia (finger necrosis)
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9
Q

Medium vessel vasculitis: 2 physical exam findings

A
  1. Livedo reticularis (net-like red-blue discoloration)
  2. Vasculitis nodules on hands
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10
Q

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

A

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

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11
Q

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

A

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

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12
Q

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

A

Anti-Neutrophil Cytoplasmic Antibodies (ANCA)

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13
Q

2 types of ANCAs

A

cANCA: cytoplasmic
pANCA: perinuclear

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14
Q

cANCAs and pANCAs are directed against 2 different proteins…

A

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

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15
Q

cANCA is associated with a specific type of vasculitis called…

A

granulomatosis with polyangiitis (GPA)

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16
Q

pANCA is associated with 2 specific types of vasculitis called…

A
  • microscopic polyangiitis (MPA)
  • eosinophilic granulomatosis with polyangiitis
17
Q

3 types of ANCA-associated vasculitis

A
  1. Granulomatosis with polyangiitis
  2. Eosinophilic granulomatosis with polyangiitis
  3. Microscopic polyangiitis
18
Q

Peak age of onset for ANCA-associated vasculitis

19
Q

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

A

b) males (1.3:1)

20
Q

Describe granulomatosis with polyangiitis (GPA)

A
  • Necrotizing granulomatous inflammation of the upper and lower respiratory tract (sinuses, nose, trachea, bronchi, lungs)
  • Necrotizing vasculitis of the small arteries and veins
21
Q

4 clinical manifestations of granulomatosis with polyangiitis (GPA)

A
  1. Upper or lower respiratory tract symptoms (see slides)
  2. Necrotizing pauci-immune glomerulonephritis (kidneys)
  3. Arthritis
  4. Purpura
22
Q

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

A
  1. Saddle-nose deformity (due to cartilage destruction)
  2. Perforated septum
  3. Eye involvement and inflammation
23
Q

Describe microscopic polyangiitis (MPA)

A
  • 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
24
Q

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

A
  1. Glomerulonephritis
  2. Pulmonary hemorrhage
  3. Mononeuritis multiplex (i.e. multiple mononeuropathies)
25
Q

How do we diagnosis microscopic polyangiitis (MPA)?

A
  • pANCA present in 50-80% of patients
  • biopsy should be obtained
26
Q

Describe eosinophilic granulomatosis with polyangiitis (EGPA)

A

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

27
Q

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

A
  • Asthma
  • Eosinophilia
  • Systemic vasculitis
28
Q

EGPA: Progression of disease (3 phases)

A
  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
29
Q

What antibody is associated with GPA?

A

cANCA (anti-PR3)

30
Q

What antibody is associated with MPA?

A

pANCA (anti-MPO)

31
Q

What antibody is associated with EGPA?

A

pANCA (anti-MPO)

32
Q

Review Key Clinical Pearls! Last slide

BLOCK F (27 decks)

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