Vasculitis

Question 1

What are the large vessel vasculitities?

Answer 1

Giant Cell Temporal Arteritis

Takayasu’s Arteritis

Question 2

Describe Giant Cell Temporal Arteritis

Answer 2

• Inflammation of the temporal artery
• Affects primarily older patients
• Present with headache, visual disturbances, and tenderness along the temporal artery
• Initiate treatment with steroids quickly as complete occlusion of the temporal artery can result in permanent blindness

Question 3

Describe Takayasu’s Arteritis

Answer 3

• AKA “Pulseless Disease”
• Inflammation/Granulomas of the aorta or its branches (carotid, subclavian, etc)
• Affects primarily young Asian women
• Present with nonspecific symptoms such as fever and night sweats

Question 4

What are the medium vessel vasculitities?

Answer 4

Kawasaki's disease
Polyarteritis Nodosa (PAN)

Question 5

Describe Kawasaki’s Disease

Answer 5

• occurs primarily in infants and children < 5
• presents with several days of high, spiking fevers, strawberry tongue and chapped lips (mucositis), and cervical LAD
• can weaken the coronary vessels and result in coronary aneurysms
• treat with IVIG and aspirin (in this case the benefits outweigh the risks)

Question 6

Describe Polyarteritis Nodosa

Answer 6

• Immune complex disease that is associated with Hep B

- can affect any organ system that is associated with medium vessels

Question 7

What are the two kinds of small vessel vasculitities?

Answer 7

• Pauci Immune/ANCA diseases

- Immune complex diseases

Question 8

What are the Pauci Immune/ANCA diseases?

Answer 8

• Wegner’s granulomatosis
• Microscopic Polyarteritis
• Churg Strauss syndrome

Question 9

What are the Immune complex vasculitities?

Answer 9

• SLE
• Henoch - Schonlein purpura
• Cryoglobinemia
• drug induced vasculitis

Question 10

Describe Wegner’s granulomatosis?

Answer 10

• associated with C ANCA
• necrotizing granulomatous vasculitis that affects the vessels of the respiratory tract or kidneys.
• vessels are destroyed, leading to death of the surrounding tissue
• presents with coughing up blood

Question 11

Describe Churgg-Straus Syndrome

Answer 11

• associated with P ANCA
• allergic granulomatous angiitis that leads to vasculitis of the pulmonary vasculature.
• presents with asthma and patients frequently have peripheral eosinophilia.

Question 12

Describe Microscopic Polyangiitis

Answer 12

• associated with P ANCA

- ill defined disease with no asthma or granulomas

Question 13

What diseases is cryoglobinemia associated with?

Answer 13

It is a small vessel vasculitis that is associated with Hep B and C.

Question 14

Describe Henoch-Schonlein purpura?

Answer 14

• bright red maculopapular rash that results when the small vessels start bleeding into the skin.

Question 15

What is ANCA?

Answer 15

ANCAs are anti-neutrophil cytoplasmic antibodies. They do not form immune complexes.
There are two types of ANCAs - P-ANCA and C-ANCA.

Question 16

What is C - ANCA?

Answer 16

C-ANCA is cytoplasmic ANCA. It’s antigen is proteinase 3, a neutrophil enzyme. It is associated with Churgg-Struass and Microscopic Polyangiitis.

Question 17

What is P - ANCA?

Answer 17

P-ANCA is peri-nuclear ANCA. It’s antigen is myeloperoxidase , a neutrophil enzyme. It is associated with Wegner’s.