Vasculitis

Question 1

Background

Answer 1

a. Caused by an immune reaction that occurs at the level of the vessel.
i. Infectous causes such as ricketssia or bacteria
ii. Non-infectous
b. Neutrophilic antibodies
i. C-ANCA – PR3 protein most commonly associated with Wegner’s
ii. P-ANCA – MPO protein associated with Microscopic poly-angitis and Churg-Strauss
c. Endothelial antibodies
i. Kawasaki Disease
d. Most commonly present with constitutional symptoms and the involvement of the specific organ. Can be highly variable between patients and within a single patient over time.
e. Treatment is generally steroids and biopsy is usually the most definitive diagnosis because there is low specificity for the tests.

Question 2

Giant Cell arteritis (temporal)

Answer 2

i. Pathology
1. Granulomatous inflammation of large and small vessels mainly of the head, especially the temporal artery.
2. Characterized by giant cells.
3. Lesions are not continuous and there can be large segments that are unaffected.
ii. Clinical
1. Constitutional symptoms with elevated ESR, Temporal artery hardening and tenderness.
2. Involvment of the ophthalmic artery leads to ocular disturbances
3. Elderly, must occur in patients over 50 years of age.
iii. Tx
1. Emergency to get steroids because of risk of blindness.

Question 3

Takayasu Arteritis (pulseless disease)

Answer 3

i. Pathology
1. Fibrous inflammation and luminal narrowing of large vessels off the aorta. Intimal hyperplasia
2. Granuloma’s present with giant cells.
ii. Clinical
1. Presents as absent or reduced pulses
2. Can lead to hypertension
3. Can lead to aortic insufficiency and also blindness.

Question 4

Polyarteritis Nodosa

Answer 4

i. Pathology
1. Small and medium sized involvement is diffuse in different vascular beds
2. Associated with Hepatitis B surface antigen.
3. Spares pulmonary but often hits renal.
4. Transmural inflammation and fibrinoid necrosis
ii. Clinical
1. Presentation is widespread and variable
2. Renal impairment and nephrotic syndrome is the most common and dangerous.

Question 5

Kawasaki Disease

Answer 5

i. Pathology
1. Unkown etiology, likely viral. Leads to anti endothelial antibodies.
2. Propensity for medium sized vessels.
ii. Clinical
1. Children. Involves the coronary arteries.
2. Children with MI is Kawasaki
3. Commonly erythema and edema of hands, feet, and mouth

Question 6

Microscopic Polyangitis

Answer 6

i. Pathology
1. Necrotizing vasculitis of capillaries and small vessels.
2. P-ANCA positive
3. All lesions are same age
ii. Clinical
1. Preferentially effect the lungs and the Kidneys. Nephrotic syndrome and pulmonary involvement.

Question 7

Churgg Straus

Answer 7

i. Pathology
1. Allergic Vasculitis
2. Eosinophils and IgE cause a granulomatous inflammation
3. Often effects heart
4. P-ANCA positive
ii. Clinical
1. In patients with a history of asthma
2. Cardiomyopathy is most common cause of death.

Question 8

Wegner’s Granulomatosis

Answer 8

i. Pathology
1. Granulomas that are positive for C-ANCA
2. Most commonly caused by inhaled substance
3. Involves the lungs and the kidney
ii. Clinical
1. Nephrotic syndrome and granulomas in the lungs

Question 9

Thromboangitis Obliterans

Answer 9

i. Pathology
1. Seen in overweight male smokers.
2. Likely from damage to vessel walls by something in smoke
3. Causes necrosis of vessel and skin