Vasculopathies

Question 1

What are the constitutional symptoms for vasculopathies?

Answer 1

fever, arthralgias/myalgias, weight loss, malaise

Question 2

necrotizing arteritis of medium-sized vessels with a predilection for involving peripheral nerves, mesenteric vessels, heart and brain

Answer 2

polyarteritis nodosa

Question 3

What is polyarteritis nodosa commonly associated with?

Answer 3

vasculitic neuropathy

Question 4

Infectious disease responsible for 10% of polyarteritis nodosa

Answer 4

Hep B

Question 5

Typical skin involvement includes: livedo reticularis, subcutaneous nodules, and skin ulcers

Answer 5

polyarteritis nodosa

Question 6

What is often the most frequent and earliest symptom of polyarteritis nodosa?

Answer 6

Peripheral neuropathy

Question 7

Would you expect the ANCA results for polyarteritis nodosa to be positive or negative?

Answer 7

ANCA negative

Question 8

What is required for diagnosis of polyarteritits nodosa?

Answer 8

tissue biopsy or angiogram

Question 9

What is the mainstay of treatment for polyarteritis nodosa?

Answer 9

high dose steroids initially. Cyclophosphamide for severe disease

Question 10

Most severe medium sized vessel disease of childhood. Asian children are at higher risk.

Answer 10

Kawasaki’s

Question 11

Classic h/o includes: high fever, conjunctival injection, mucositis, erythematous rash, and enlarged cervial lymph nodes

Answer 11

Kawasaki’s

Question 12

What is a major complication of Kawasaki’s?

Answer 12

arteritis of the coronary vessels

Question 13

Should be performed as soon as Kawasaki’s is suspected, at 2 weeks, and at 6-8 weeks after onset of disease

Answer 13

echocardiogram

Question 14

Recommended to evaluate arrhythmias, prolonged PR interval, and nonspecific ST and T wave changes of Kawasak’s

Answer 14

EKG and echocardiogram

Question 15

Patient population with Kawasaki’s more likely to develop coronary artery aneuryms

Answer 15

infants

Question 16

Affects small arteries. Particularly in the upper and lower respiratory tract, and kidneys. Commonly occurs in the fourth and fifth decade

Answer 16

Wegener’s Granulomatosis

Question 17

Percentage of patients with Wegener’s Granulamatosis that present with upper or lower respiratory tract symptoms

Answer 17

90%

Question 18

serum antibody test that is usually elevated with Wegener’s Granulomatosis

Answer 18

C-ANCA

Question 19

Most common vasculitis in children. Affects small vessels.

Answer 19

Henoch-Schonlein Purpura

Question 20

Typical features of Henoch-Schonlein Purpura

Answer 20

palpable purpura, abdominal pain, arthritis, hematuria/proteinuria

Question 21

How soon do most cases of Henoch-Shonlein Purpura resolve?

Answer 21

within one month

Question 22

What is the main cause of morbidity associated with Henoch-Schonlein Purpura?

Answer 22

renal disease

Question 23

How frequently should you follow-up a patient with Henoch-Schonlein purpura with UA and BP monitoring?

Answer 23

Weekly for first two months, then monthly or every other month for one year