Local Autoimmune Vasculitis:

-temporal arteritis
-Takayasu arteritis
-Thromboangiitis obliterans (Buerger disease)

Systemic Autoimmune Vasculitis- more severe

-Polyarteritis nodosa
-Kawasaki disease
-Microscopic polyangiitis
-Wegner granulomatosis

1. Temporal arteritis (giant cell arteries)

Chronic granulomatous inflammation of large and medium-
sized arteries
Granulomas in the in the temporal and cranial arteries
T-cell mediated
Type IV hypersensitivity
Women at 70 years of age most at risk

Fever
Markers of inflammation increased
Increased risk for stroke and permanent blindness

Treatment: high-dose steroids and aspirin

2. Takayasu Arteritis
Granulomatis inflammation of the aorta
Generally in younger women
Scarring and fibrous obliteration of main branches off aorta
 Diagnosis: imaging to see inflamed and distended aorta

3. Thromboangiitis obliterans (Buerger Disease)

Small and medium-sized arteries in the hands and feet
Almost exclusively in heavy smokers

Vascular occlusion leads to painful ulcers or gangrene of

the digits

4. Polyarterities Nodosa
Autoimmune
Idiopathic
Small and medium-sized muscular arteries (NOT veins)
Necrotizing inflammationàmicroanyeurysms due to
inflammation in vesselsàischemia or infarction in kidneys
and viscera

5. Kawasaki Disease
Autoimmune
Idiopathic
Coronary artery affected, in addition to lymph nodes, skin
and mucous membranes
An important cause of acquiredheart disease in
CHILDREN!! (6months to 5 years old)

Treatment: high-dose aspirin and IVIG

Type II hypersensitivity

6. Microscopic Polyangiitis (hypersensitivity vasculitis)

Autoimmune condition that causes small blood vessels to
be inflammed
Idiopathic
Glomeruli and pulmonary capillaries primarily affected
Associated with penicillin
 Type II hypersensitivity in response to penicillin med use

7. Wegner Granulomastosis
Granulomatosis with Polyangiitis
Type II hypersensitivity of the small and medium-sized
arteries
Affecting primarily nose, throat, sinuses, lungs, and
kidneys

Clinical signs of the systemic autoimmune vasculitis

-malaise
-fever
-muscle aches
-joint pain

Treatment: steroids and immunosuppressants, IVIG

NON-Autoimmune Vasculitis
Usually local and due to bacterial or fungal infection
Infection weakens the wall, creating an aneurysm or can induce
thrombosis and infarction

1. Raynaud’s Phenomenon
Exaggerated vasomotor reactivity in the small arteries and
arterioles in the hands and fingers (extremities of body)
-triggered by cold or emotional stress
Typically one or more fingers blanch at the tip when
exposed to cold, fingers may then turn blue
-when warmed, the area becomes hyperemic
Primary occurs with no underlying disease (80%)
Secondary mostly associated with an autoimmune disorder

Treatment: avoidance of triggers, especially smoking,

 vasodilators may be helpful

2. Varicose Veins
Abnormally dilated veins due to incompetent valves
secondary to age-related relaxation of supporting tissue
-Superficial veins are more often affected as they are
surrounded by less supporting tissue
-Most patients over 50, obese, or are required to stand for
long periods of time
-Common in pregnant women as well due to increased
pelvic veins reducing venous return

-Treatment: compression stockings (to help support over

compliant veins in venous return) or surgical excision

3. Hemorrhoids
Varicose veins of the anus
-Treatment: anti-inflammatories may offer temporary relief
but sclerotherapy or surgery may be required

4. Esophageal Varices
Varicose veins of the esophagus
-occur with cirrhosis of the liver as portal venous blood
flow through the liver is obstructed and increases pressure
-can rupture and caused fatal gastrointestinal bleeding

**Due to hypercompliance of veins, lead to pooling of blood in

those veins**

Thrombophlebitis:
Formation of venous thrombi
-90% cases occur in deep veins of the legs (DVT)
-may embolize the lungs (PE)
-Treatment: thrombolytic drugs, anticoagulants or surgery

-Risk factors: anything that causes increased venous pressure or

sluggish blood flow

TUMORS OF BLOOD AND LYMPHATIC VESSELS

Hemangiomas:
Common and usually benign
Usually in skin that appear erythematous and raised (port wine
stains, nevus flammeus)
Most resolve on their own
Treatment: steroids or antihypertensives may be used

Spider hemangiomas:
Acquired, bright red vascular growth
Related to elevated estrogen levels in the body
Common in pregnant women or patients with cirrhosis who are
unable to metabolize and excrete estrogen
-regress if estrogen levels decline

Cavernous hemangiomas:
Comprised of larger vessels
Tend to occur in deeper tissue such as the brain and liver
Usually remain clinically silent but may bleed spontaneously

Lymphangiomas:
Benign, abnormal collection of lymphatic capillaries
Less common than hemangiomas
Almost always in SQ head and neck

Kaposi sarcoma (KS):

Malignant vascular tumor most often seen in patients with
AIDS but can be seen in other immunodeficiencies
-Caused by human herpes vius-8
-Small purple plaques or nodules
-aggressive varieties may enlarge into fleshy, bloody masses in
lymph nodes or organs
-treatment: antiretrovirals, chemotherapy, radiation and
cryotherapy

Angiosarcoma:
Rare malignant tumor of vascular endothelial cells
-most often occurs in skin, breast tissue, soft tissue and liver
-Treatment: combination of surgery, chemotherapy and radiation