ANCA associated vasculitis and medium vessel vasculitis

Question 1

What are the three ANCA associated vasculitis?

Answer 1

1. Polyangiitis with granulomatous (Wegners)
2. Microscopic polyangiitis
3. Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss)

Question 2

What are the three ANCA associated vasculitis?

Answer 2

1. Polyangiitis with granulomatous (Wegners)
2. Microscopic polyangiitis
3. Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss)

Question 3

What are the ANCAs for :

1. Polyangiitis with granulomatous (Wegners)
2. Microscopic polyangiitis
3. Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss

Answer 3

1. C-ANCA
2. P-ANCA
3. P-ANCA

Question 4

Triad of Granulomatosis with polyangiitis:

Answer 4

• Upper respiratory symptoms (severe sinusitis, oral ulcerations, strawberry gums)
• lower respiratory symptoms (cough, SOB, hemoptysis)
• renal changes (severe glomerulonephritis w/ hematuria

Question 5

Skin findings in Granulomatosis with polyangiitis:

Answer 5

• Palpable purpura
• pyoderma gangrenosum-like ulcers
• Sub-Q nodules

Question 6

Histology of Granulomatosis with polyangiitis:

Answer 6

LCV w/ necrotizing palisading granulomas

Question 7

Tx of Granulomatosis with polyangiitis:

Answer 7

Immosuppresants (cyclophosphamide, pred, MTX, rituximab)

Question 8

Microscopic polyangiitis clinical presentation:

Answer 8

pulmonary-renal syndrome (lower respiratory+glomerulonephritis)

***basically like Wegner’s but without upper respiratory symptoms, and is P-ANCA

Question 9

Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss) clinical findings:

Answer 9

1. Adult onset asthma, allergic rhinitis, and nasal polyps
2. Eosinophilia, pneumonia, GI issues
3. Systemic vasculitis w/ palpable purpura

note: no renal disease (distinguishes from the other two
ANCA associated vasculitis)

Question 10

Polyarteritis nodosa clinical presentation:

Answer 10

• Skin: palpable purpura, painful-subQ nodules, lived reticular
• constitutional sx
• Neurologic changes (paresthesias, motor neuropathies, foot drop!)
• other systemic symptoms: cardiac arrhythmia, bowel infarction→GI hemorrhage, renal failure, HTN

Question 11

Why do polyarteritis nodosa patients not get glomerulonephritis?

Answer 11

Because it affects medium sized vessels that perfuse the kidney (not the small vessels within the glomeruli)

Question 12

Assocations of polyarteritis nodosa:

Answer 12

• Hep B+C
• HIV
• CMV
• strep
• IBD

Question 13

Histology of polyarteritis nodosa:

Answer 13

LCV w/ medium-sized arteries

or

Sub-Q +/- lobular panniculitis next to involved vessels

Question 14

Kawasaki disease clinical criteria:

Answer 14

CRASH & BURN

Fever + 4/5 of the following:

• conjunctivitis
• rash (polymorphous exanthem)
• Adenopathy
• Strawberry tongue
• Hand and feet desquamation
• Burning fever for 5+ days

Question 15

Labs for Kawasaki disease:

Answer 15

WATCH Echo

• WBC elevation
• Anemia
• Thrombocytosis/thrombocytopenia
• CRP elevation
• Hypoalbuminemia
• STAT echo to check for coronary artery aneurysms

Question 16

When do coronary artery aneurysms present in Kawasaki disease?

Answer 16

several weeks after sx onset in 25% of untreated patients

Question 17

Tx of Kawasaki disease:

Answer 17

IVIG

Aspirin 80-100mg qd