Vasculitis

Question 1

What is vasculitis

Answer 1

Inflammatory destruction of blood vessels.
MOA: Leukocyte migration causes vessel destruction. Arteritis or phlebitis.

Can be primary (80%, no known cause) or secondary (20%, due to autoimmune disease)

20 different types

Question 2

Primary vasculitis (3)

Answer 2

80%
No known cause

1. Giant cell arteritis/Temporal arteritis (40%)
2. Polyarteritis Nodosa (5%)
3. Granulomatosis with polyangitis (3.4%)

Question 3

Secondary vasculitis (2)

Answer 3

20% due to autoimmune disease

1. SLE (3.7%)
2. Behcet disease (less than 1%)

Question 4

Main 3 vasculitis signs

Answer 4

1. Palpable purpura- small raise hemorrhages, rash on skin.
2. Reduced visual acuity
3. Acute visual loss

Question 5

Non specific labs/tests for vasculitis

Answer 5

Sedimentation rate (ESR) 
-Faster settle= higher inflammation 
C reactive protein from liver 

Other: Anti neutrophil cytoplasmic Ab (ANCA)

Question 6

Giant cell arteritis/temporal arteritis

Answer 6

Large and medium vessels affected
-If posterior ciliary arteries affected, can cause ischemic optic neuropathy.

Disease of the elderly. 70-80%
F>M, White> AA, asian.

Headaches
Jaw Claudication
Scalp tenderness/temporal artery tenderness.

Idiopathic

Can cause irreversible blindness, MI, stroke, or death.

Question 7

Dx of giant cell arteritis

tx

Answer 7

+ESR and +CPR
97% specificity

3 characteristic symptoms:
Headaches
Jaw Claudication
Scalp tenderness/temporal artery tenderness

TX: immediate oral steroids. 80mg pred

Question 8

Ocular complications of GCA

Answer 8

Some form of vision loss (90%)
Ischemic optic neuropathy (80%)
-Anterior more common than posterior
Amaurosis fugal

1/5 have vision loss WITHOUT systemic symptoms.

Question 9

Polyarteritis Nodosa (primary)

Answer 9

Small and medium arteries, necrotizing inflammation. 
Idiopathic, maybe associated with hep B 
Rare, M>F. Onset 40-60.
May cause peripheral neuropathy 
Lungs are not involved !!!!!!
ANCA is negative. ESR may be high. 
20% ocular involvement. Scleritis

Question 10

Granulomatosis with polyangitis (wegener’s)

Answer 10

Idiopathic 
Small and medium vessel 
Upper respiratory tract (70%) 
Lower respiratory tract (80%) AKA lungs 
Kidneys (80%) 
Onset 50 yrs 
White >> other 

DX:
(+) ANCA, (+) Biopsy
Fatal if untreated within 2 years. Kidney failure.

Ocular involvement (40%)
-Orbital pseudotumor (30%) May cause diplopia or reduced acuity.

Question 11

SLE

Answer 11

Type III hypersensitivity 
Chronic, systemic autoimmune CT disorder. 
Systemic inflammation. Joints, kidneys, lung, heart, brain. 
Mainly skin and joins- butterfly rash.
Idiopathic.
F>>M. 20-40 year onset. 
AA> Asian> white 
Dx: (+) ANA 95%

Question 12

SLE eye involvement

Answer 12

33% of cases have eye involvement

Dry eye is most common, then diplopia.

Question 13

Behcet’s disease

Answer 13

Autoimmune. Preference for veins.
100% mouth sores
70% joint pain
60% genital ulcers

20-40 year old. M>F
Rare, but 20% mortality over 7 years.

EYE involvement (60%)

• Retinal phlebitis 80%
• Uveitis 60%

HLA b51 72-79%
Bechcetine pathergy test 79%

Ocular triad:
Iritis with hypopyon
Mouth sores
Genital sores