Vasculitis

Question 1

All of these ANCA-associated vasculitides affect small- to medium-sized blood vessels and tend to be associated with the presence of autoantibodies to antigenic proteins normally present in the granules of neutrophils (name 3)

Answer 1

GPA, MPA, EGPA

Question 2

Clinical signs of this disease of the large vessels includes 3 out of 5 of the following:

• Age > 50
• New onset headache
• ESR > 50
• Abnormal artery biopsy
• Temporal artery abnormality

Answer 2

Giant cell arteritis

*ESR is the rate at which red blood cells sediment in a period of one hour. It is a common hematology test, and is a non-specific measure of inflammation.

Question 3

This disease may cause visual loss, jaw claudication (pain due to ischemia) and scalp tenderness - histo looks like this:

Answer 3

Giant cell arteritis

Question 4

treatment for giant cell arteritis?

Answer 4

corticosteroids (+ bisphosphonates to preserve bone density)

Question 5

name the disease:

• does not have ANCA
• strong association with Hep B surface antigen
• mononeuritis multiplex and hypertension

Answer 5

polyarteritis nodosa (PAN

*this is a medium vessel vasculitis and it tends to affect larger rather than smaller vessels (ie, HTN more common than GN)

Question 6

patients presenting with this disease may have sinusitis, pneumonitis (nodules that cavitate), and antibodies to proteinase 3; renal involvement in 85% of patients

Answer 6

granulomatosis with polyangitis (GPA)

*note the classic crescentic glomerulonephritis

Question 7

asthma is the cardinal feature of this vascular disease, in 90% of patients; neuropathy and sinus involvement is also very common

Answer 7

eosinophilic granulomatous polyangitis (EGPA)

Question 8

Though it is not a sensitive or specific serologic test, in eosionphilic granulomatous polyangitis _-ANCAs are found against _______.

Answer 8

P-ANCA; myeloperoxidase

Question 9

a rare disease that is characterized by rapidly progressing GN and weight loss, sometimes with pulmonary hemorrhage, and is sometimes associated with P-ANCA

Answer 9

microscopic polyangitis (MPA)

Question 10

this drug caused mortality in vasculitides to drop from 90% to 5% and is used therapeutically to induce remission

Answer 10

cyclophosphamide

*toxicity is a concern

Question 11

this is a disease of young people with small vessel involvement and classically presents with:

• palpable purpura
• abdominal pain
• renal disease

Answer 11

IgA vasculitis

Question 12

this is the most common systemic vasculitis in children (ages 3-15) and up to half of cases are preceded by URI

Answer 12

IgA vasculitis

Question 13

therapy for IgA vasculitis?

Answer 13

corticosteroids; prednisone in the event of renal disease

Question 14

Which of these is NOT one of the 4 classic clinical manifestations of Henoch Schonlein Purpura (IgA vasculitis)?

A. Purpura

B. Abdominal pain

C. Renal involvement

D. Pneumonitis

E. Arthritis/Arthralgia

Answer 14

D

Question 15

Which of the following organ systems is NOT commonly involved in PAN?

A. Renal

B. Neurologic

C. Pulmonary

D. Musculoskeletal

Answer 15

C.

Pulmonary system involvement is characteristic of GPA.