Vasculitis

Question 1

Infectious causes of vasculitis

Answer 1

Fungal (Aspergillus), Bacterial (Pseudomonas), Viral (CMV)

Question 2

Epidemiology of Temporal (Giant Cell) Arteritis

Answer 2

Elderly (>50) white females of Northern European ancestry

Question 3

Why must you be elderly to get Temporal (Giant Cell) Arteritis?

Answer 3

Giant cells attack only CALCIFIED internal elastic lamina in arteries

Question 4

Most severe complication of Temporal (Giant Cell) Arteritis?

Answer 4

Blindness (why you must make the dx)

Question 5

Symptoms/Signs of Temporal (Giant Cell) Arteritis?

Answer 5

Headache, Swollen tender artery, visual disturbances, jaw claudication

Question 6

Main immune cells involved in Temporal Arteritis?

Answer 6

Dendritic Cells and Th1 cells

Question 7

Tx for Temporal Arteritis?

Answer 7

Corticosteroids and anti-TNF therapy, almost complete reduction in Th17 cells

Question 8

MMPs up-regulated in Temporal Arteritis?

Answer 8

MMP-2 and MMP-9, destroy the elastin

Question 9

What must you know about doing a biopsy for Temporal Arteritis?

Answer 9

The disease can be segmental so false negatives are possible (commonly only lymphocytic on biopsy)

Question 10

Blood test important for dx of Temporal Arteritis?

Answer 10

ESR, normally >100

Question 11

Epidemiology of Takayasu Arteritis?

Answer 11

Young east Asian females and children

Question 12

Most important clinical manifestations of Takayasu Arteritis?

Answer 12

Pulseless upper extremity, difference in bilateral blood pressures, visual defects

Question 13

Pathogenesis of Takayasu Arteritis?

Answer 13

Necrotizing granulomatous vasculitis of the Aortic Arch and its vessels–> Aortic tear, dissection, or rupture

Question 14

Cardiac complication of Takayasu Arteritis?

Answer 14

Cardiac Tamponade

Question 15

Tx for Takayasu Arteritis?

Answer 15

Corticosteroids and Cytotoxic immunosuppression

Question 16

Epidemiology of Polyarteritis Nodosa?

Answer 16

White males in their 40s

Question 17

Important presentation of Polyarteritis Nodosa?

Answer 17

Neuropathy (sensory or motor like wrist/foot drop), Renal involvement with HTN

Question 18

Effect of Polyarteritis Nodosa on the Lungs?

Answer 18

None

Question 19

Skin change seen with Polyarteritis Nodosa?

Answer 19

Livedo Reticularis (mottled discoloration of the skin)

Question 20

Most distinguishing microscopic characteristic of Polyarteritis Nodosa?

Answer 20

Fibrinoid Necrosis, neutrophilic in the acute phase

Question 21

HBsAg (Hepatitis B) is associated with? (not diagnostic)

Answer 21

Polyarteritis Nodosa

Question 22

Tx of polyarteritis Nodosa?

Answer 22

Corticosteroids and cytotoxic immunosuppression (if necessary)

Question 23

Angiogram appearance of Polyarteritis Nodosa?

Answer 23

“Beads on a String”, focal vasculitis producing nodular lesions

Question 24

Epidemiology of Kawasaki Disease?

Answer 24

East Asian children < 5 (usually presents around age 1)

Question 25

Symptoms/Signs of Kawasaki Disease?

Answer 25

Polymorphous rash, cervical lymphadenopathy, erythema/edema/desquamination of hands and feet (KNOW THIS), and “strawberry tongue” glossitis (KNOW THIS)

Question 26

Pathophysiology of Kawasaki Disease?

Answer 26

Necrotizing vasculitis of the coronary arteries, patients get coronary aneurysms, MIs, and SCD if untreated

Question 27

Tx for Kawasaki Disease?

Answer 27

ASA and IVIG

Question 28

What is Reye syndrome?

Answer 28

Rare condition causing cerebral edema and hepatic steatosis when taking ASA for viral illness (he didn’t teach us this, but “Reye Syndrome” is in his slides)

Question 29

Prognosis for Kawasaki Disease?

Answer 29

Good if treated, most aneurysms resolve

Question 30

Pathophysiology of Microscopic Polyangiitis?

Answer 30

Necrotizing fibrinoid vasculitis of capillaries, small arterioles, and venules (mostly glomeruli and pulmonary capillaries)

Question 31

How can you differ Microscopic Polyangiitis from Wegner’s granulomatosis?

Answer 31

Microscopic Polyangiitis has no nasopharyngeal involvement

Question 32

Antibodies seen in Microscopic Polyangiitis?

Answer 32

p-ANCA (anti-Myeloperoxidase)

Question 33

Epidemiology of Wegner’s Granulomatosis?

Answer 33

Whites in their 40s, men and women equally affected

Question 34

Pathophysiology of Wegener’s Granulomatosis?

Answer 34

Necrotizing granulomatous vasculitis of arteries and veins in the respiratory tract (+ hemoptysis) and kidneys

Question 35

Common findings of physical exam for Wegener’s Granulomatosis?

Answer 35

Mucosal ulcers, nasal septal cartilage destruction, and other nasopharyngeal lesions (distinguishes it from micorscopic polyangiitis)

Question 36

Tx of Wegener’s Granulomatosis?

Answer 36

Corticosteroids and Rituximab (B cell involvement)

Question 37

Triad seen in Churg Strauus Syndrome?

Answer 37

Asthma, Vasculitis, and Eosinophila (KNOW THIS)

Question 38

Antibody seen in Churg Strauss Syndrome?

Answer 38

p-ANCA (not dx)

Question 39

Epidemiology of Buerger Disease?

Answer 39

Young male smokers of Middle Eastern and South Asian ancestry

Question 40

Pathophysiology of Buerger Disease?

Answer 40

inflammatory thrombosing vaso-occlusive disease of arteries/veins of the limbs

Question 41

Tx for Buerger disease?

Answer 41

Stop smoking and amputation for gangrene

Question 42

Pathophysiology of peripheral arterial disease?

Answer 42

Chronic atherosclerotic occlusive disease of large/medium arteries of the legs

Question 43

Symptoms of peripheral arterial disease?

Answer 43

Intermittent claudication (leg pain with exercise, relieved by rest). Mainly in the calf muscles (femoral/popliteal arteries). Ulcers and pain at rest–> severe disease

Question 44

The signs/symptoms of peripheral arterial disease (the 5 Pee Pees)

Answer 44

Pain, pallor, paralysis, paresthesia, and pulselessness

Question 45

Tx for peripheral arterial disease?

Answer 45

Exercise, especially walking. Helps to develop collateral perfusion

Question 46

Tx for acute arterial occlusion?

Answer 46

Emergency surgery